Exercise in muscle glycogen storage diseases.

Science.gov (United States)

Preisler, Nicolai; Haller, Ronald G; Vissing, John

2015-05-01

Glycogen storage diseases (GSD) are inborn errors of glycogen or glucose metabolism. In the GSDs that affect muscle, the consequence of a block in skeletal muscle glycogen breakdown or glucose use, is an impairment of muscular performance and exercise intolerance, owing to 1) an increase in glycogen storage that disrupts contractile function and/or 2) a reduced substrate turnover below the block, which inhibits skeletal muscle ATP production. Immobility is associated with metabolic alterations in muscle leading to an increased dependence on glycogen use and a reduced capacity for fatty acid oxidation. Such changes may be detrimental for persons with GSD from a metabolic perspective. However, exercise may alter skeletal muscle substrate metabolism in ways that are beneficial for patients with GSD, such as improving exercise tolerance and increasing fatty acid oxidation. In addition, a regular exercise program has the potential to improve general health and fitness and improve quality of life, if executed properly. In this review, we describe skeletal muscle substrate use during exercise in GSDs, and how blocks in metabolic pathways affect exercise tolerance in GSDs. We review the studies that have examined the effect of regular exercise training in different types of GSD. Finally, we consider how oral substrate supplementation can improve exercise tolerance and we discuss the precautions that apply to persons with GSD that engage in exercise.

Lysosomal storage diseases: current diagnostic and therapeutic options

International Nuclear Information System (INIS)

Malinova, V.; Honzik, T.

2013-01-01

Lysosomal storage diseases are rare genetic diseases caused by insufficient activity of some of the lysosomal enzymes and/or transport proteins. Initial symptoms may appear any time from the neonatal period to late adulthood; early forms tend to have a severe course with rapid progression and unfavorable prognosis. There is multisystem involvement with continuous progression of symptoms and involvement of metabolically active organs or tissues – the bone marrow, liver, bones, skeletal muscles, myocardium, or CNS. The diagnosis is definitively confirmed by demonstration of reduced activity of the particular enzyme and by mutation analysis. Some of the storage diseases can be effectively treated by intravenous administration of recombinant enzymes or by limiting the amount of the substrate stored. In a small number of lysosomal storage diseases, bone marrow transplantation is successful. Multidisciplinary collaboration, including genetic counselling and prenatal diagnosis in patient families, is required. The first part of the paper deals with general characteristics of lysosomal storage diseases and the most common diseases that are currently treatable in the Czech Republic (Gaucher’s disease, Pompe disease, Fabry disease, Niemann–Pick disease, cholesterol ester storage disease). The second part of the paper deals with mucopolysaccharidase, another group of rare lysosomal storage diseases. (author)

Gaucher disease and other storage disorders.

Science.gov (United States)

Grabowski, Gregory A

2012-01-01

In 1882, Philippe Gaucher described a 32-year-old woman with massive splenomegaly and unusually large cells in the spleen, which he called a "primary epithelioma of the spleen." The systemic nature and inheritance of the disease and its variants involving the viscera and CNS were described over the next century. The delineation of the causal enzymatic defects, genetics, molecular pathology, and genomics have provided pathogenic insights into the phenotypic spectrum and the bases for development of specific therapies for what is now known as Gaucher disease. As a prototype, the clinically and economically successful intracellular enzyme therapy provided the impetus for the expansion of similar research and therapeutic developments for other lysosomal storage diseases (LSDs) and orphan diseases, including Fabry, Pompe, and Niemann-Pick diseases, as well as several mucopolysaccharidoses. Continuing studies of such LSDs, which occur as a group in more than 7000 live births, have revealed the complex molecular interdigitation with the autophagy and apoptotic pathways and proteostasis and the impact of disruptions of the lysosomal/autophagy and proteostasis systems on more common diseases has been recognized. Examples include age-related neurodegenerative diseases (eg, Parkinson disease and Gaucher disease), idiopathic hypertrophic myocardiopathies, stroke and renal failure (eg, Fabry disease), and Nonalcoholic Fatty Liver Disease/Nonalcoholic SteatoHepatitis (NAFLD/NASH) and atherosclerosis (eg, lysosomal acid lipase deficiencies). Although perceived as rare, the availability of treatment and the impact of the LSDs on more common diseases require their integration into routine clinical practice.

Adult onset glycogen storage disease type II (adult onset Pompe disease): report and magnetic resonance images of two cases

International Nuclear Information System (INIS)

Del Gaizo, Andrew; Banerjee, Sima; Terk, Michael

2009-01-01

Glycogen storage disease type II (GSDII), also referred to as Pompe disease or acid maltase deficiency, is a rare inherited condition caused by a deficiency in acid alpha-glucosidase (GAA) enzyme activity. The condition is often classified by age of presentation, with infantile and late onset variants (Laforet et al. J Neurology 55:1122-8, 2000). Late onset tends to present with progressive proximal muscle weakness and respiratory insufficiency (Winkel et al. J Neurology 252:875-84, 2005). We report two cases of biopsy confirmed adult onset GSDII, along with key Magnetic Resonance (MR) images. (orig.)

Lysosomal lipid storage diseases.

Science.gov (United States)

Schulze, Heike; Sandhoff, Konrad

2011-06-01

Lysosomal lipid storage diseases, or lipidoses, are inherited metabolic disorders in which typically lipids accumulate in cells and tissues. Complex lipids, such as glycosphingolipids, are constitutively degraded within the endolysosomal system by soluble hydrolytic enzymes with the help of lipid binding proteins in a sequential manner. Because of a functionally impaired hydrolase or auxiliary protein, their lipid substrates cannot be degraded, accumulate in the lysosome, and slowly spread to other intracellular membranes. In Niemann-Pick type C disease, cholesterol transport is impaired and unesterified cholesterol accumulates in the late endosome. In most lysosomal lipid storage diseases, the accumulation of one or few lipids leads to the coprecipitation of other hydrophobic substances in the endolysosomal system, such as lipids and proteins, causing a "traffic jam." This can impair lysosomal function, such as delivery of nutrients through the endolysosomal system, leading to a state of cellular starvation. Therapeutic approaches are currently restricted to mild forms of diseases with significant residual catabolic activities and without brain involvement.

Phytochemical profile of Orthosiphon aristatus extracts after storage: Rosmarinic acid and other caffeic acid derivatives.

Science.gov (United States)

Chua, Lee Suan; Lau, Cher Haan; Chew, Chee Yung; Ismail, Nurul Izzati Mohd; Soontorngun, Nitnipa

2018-01-15

Orthosiphon aristatus (Blume) Miq. is a medicinal herb which is traditionally used for the treatment of diabetes and kidney diseases in South East Asia. Previous studies reported higher concentration of antioxidative phytochemicals, especially rosmarinic acid (ester of caffeic acid) and other caffeic acid derivatives in this plant extract than the other herbs such as rosemary and sage which are usually used as raw materials to produce rosmarinic acid supplement in the market. The phytochemical profile of O. aristatus was investigated at different storage durations for quality comparison. The phytochemicals were extracted from the leaves and stems of O. aristatus using a reflux reactor. The extracts were examined for total phenolic and flavonoid contents, as well as their antioxidant capacities, in terms of radical scavenging, metal chelating and reducing power. The phytochemical profiles were also analyzed by unsupervised principal component analysis and hierarchical cluster analysis, in relation to the factor of storage at 4 °C for 5 weeks. The leaf extract was likely to have more phytochemicals than stem extract, particularly caffeic acid derivatives including glycosylated and alkylated caffeic acids. This explains higher ratio of total phenolic content to total flavonoid content with higher antioxidant capacities for the leaf extracts. Rosmarinic acid dimer and salvianolic acid B appeared to be the major constituents, possibly contributing to the previously reported pharmacological properties. However, the phytochemical profiles were found changing, even though the extracts were stored in the refrigerator (4 °C). The change was significantly observed at the fifth week based on the statistical pattern recognition technique. O. aristatus could be a promising source of rosmarinic acid and its dimer, as well as salvianolic acid B with remarkably antioxidant properties. The phytochemical profile was at least stable for a month stored at 4 °C. It is likely to be

Free Fatty Acid Storage in Human Visceral and Subcutaneous Adipose Tissue

Science.gov (United States)

Ali, Asem H.; Koutsari, Christina; Mundi, Manpreet; Stegall, Mark D.; Heimbach, Julie K.; Taler, Sandra J.; Nygren, Jonas; Thorell, Anders; Bogachus, Lindsey D.; Turcotte, Lorraine P.; Bernlohr, David; Jensen, Michael D.

2011-01-01

OBJECTIVE Because direct adipose tissue free fatty acid (FFA) storage may contribute to body fat distribution, we measured FFA (palmitate) storage rates and fatty acid (FA) storage enzymes/proteins in omental and abdominal subcutaneous fat. RESEARCH DESIGN AND METHODS Elective surgery patients received a bolus of [1-14C]palmitate followed by omental and abdominal subcutaneous fat biopsies to measure direct FFA storage. Long chain acyl-CoA synthetase (ACS) and diacylglycerol acyltransferase activities, CD36, fatty acid-binding protein, and fatty acid transport protein 1 were measured. RESULTS Palmitate tracer storage (dpm/g adipose lipid) and calculated palmitate storage rates were greater in omental than abdominal subcutaneous fat in women (1.2 ± 0.8 vs. 0.7 ± 0.4 μmol ⋅ kg adipose lipid−1 ⋅ min−1, P = 0.005) and men (0.7 ± 0.2 vs. 0.2 ± 0.1, P < 0.001), and both were greater in women than men (P < 0.0001). Abdominal subcutaneous adipose tissue palmitate storage rates correlated with ACS activity (women: r = 0.66, P = 0.001; men: r = 0.70, P = 0.007); in men, CD36 was also independently related to palmitate storage rates. The content/activity of FA storage enzymes/proteins in omental fat was dramatically lower in those with more visceral fat. In women, only omental palmitate storage rates were correlated (r = 0.54, P = 0.03) with ACS activity. CONCLUSIONS Some adipocyte FA storage factors correlate with direct FFA storage, but sex differences in this process in visceral fat do not account for sex differences in visceral fatness. The reduced storage proteins in those with greater visceral fat suggest that the storage factors we measured are not a predominant cause of visceral adipose tissue accumulation. PMID:21810594

Type I Glycogen Storage Disease

Science.gov (United States)

... the most common form of glycogen storage disease, accounting for 25% of all cases. It is an ... Links Videos Webinars About ALF OVERVIEW Programs About Liver Disease Ask the Experts People ALF ...

Lysosomal Storage Diseases To date

OpenAIRE

HOFFMANN, Björn; MAYATEPEK, Ertan

2011-01-01

New therapeutic options and progress of approved therapies have made Lysosomal Storage Diseases (LSDs) one of the most exciting group of diseases. This review aims to summarize current achievements in these particular disorders and to give an outlook towards possible future treatment options. Enzyme replacement therapy is the gold standard for Gaucher disease, Fabry disease, Mucopolysaccharidosis type I, II, and VI, and for Pompe disease. Besides this, substrate reduction has been approved fo...

Liver and Skin Histopathology in Adults with Acid Sphingomyelinase Deficiency (Niemann-Pick Disease Type B)

OpenAIRE

Thurberg, Beth L.; Wasserstein, Melissa P.; Schiano, Thomas; O’Brien, Fanny; Richards, Susan; Cox, Gerald F.; McGovern, Margaret M.

2012-01-01

Acid sphingomyelinase deficiency (ASMD) is a lysosomal storage disorder characterized by the pathologic accumulation of sphingomyelin in multiple cells types, and occurs most prominently within the liver, spleen and lungs, leading to significant clinical disease. Seventeen ASMD patients underwent a liver biopsy during baseline screening for a Phase 1 trial of recombinant human acid sphingomyelinase (rhASM) in adults with Niemann-Pick disease type B. Eleven of the 17 were enrolled in the trial...

Type I Glycogen Storage Disease

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... Legacy Society Make Gifts of Stock Donate Your Car Personal Fundraising Partnership & Support Share Your Story Spread the Word Give While You Shop Contact Us Donate Now Glycogen Storage Disease Type ...

Magnetic resonance imaging of iron storage diseases

International Nuclear Information System (INIS)

Yoshida, Hideo; Mano, Isamu; Asai, Sae; Yashiro, Naofumi; Itai, Yuji; Iio, Masahiro.

1985-01-01

We presented MRI findings of four patients of iron storage diseases with hemochromatosis and hemosiderosis. We examined detectavility of iron deposits with in vitro MR and X-CT observations of ferric (Fe 3+ ) solutions. Conculusion are as follows, 1) In detection of small amount of iron deposits, MRI is much better than X-CT. 2) MRI is a unique technique to detect iron deposits in bone marrow. 3) Early estimation of iron storage diseases will be promising using MRI technique. (author)

Lauric Acid Hybridizing Fly Ash Composite for Thermal Energy Storage

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Dawei Xu

2018-04-01

Full Text Available Fly ash includes different mineral phases. This paper reported on the preparation of a novel lauric acid (LA/fly ash (FA composite by vacuum impregnation as a form-stable phase change material (PCM for thermal energy, and especially investigated the effect of the hydrochloric acid-treated fly ash (FAh on the thermal energy storage performance of the composites. The morphology, crystalline structure, and porous textures of the samples were characterized by scanning electron microscopy (SEM, X-ray diffraction (XRD, Brunauer–Emmett–Teller (BET, X-ray fluorescence (XRF, and differential scanning calorimetry (DSC. The results indicated that hydrochloric acid treatment was beneficial to the increase of loading capacity and crystallinity of LA in the LA/FAh composite, which caused an enhanced thermal storage capacity with latent heats for melting and freezing of LA/FAh (80.94 and 77.39 J/g, higher than those of LA/FA (34.09 and 32.97 J/g, respectively. Furthermore, the mechanism of enhanced thermal storage properties was investigated in detail.

78 FR 15753 - Maintenance, Testing, and Replacement of Vented Lead-Acid Storage Batteries for Nuclear Power Plants

Science.gov (United States)

2013-03-12

...-Acid Storage Batteries for Nuclear Power Plants AGENCY: Nuclear Regulatory Commission. ACTION: Draft...-Acid Storage Batteries for Nuclear Power Plants.'' The draft guide describes methods that the NRC staff..., testing, and replacement of vented lead-acid storage batteries in nuclear power plants. DATES: Submit...

Experimental study on thermal storage performance of binary mixtures of fatty acids

Science.gov (United States)

Yan, Quanying; Zhang, Jing; Liu, Chao; Liu, Sha; Sun, Xiangyu

2018-02-01

We selected five kinds of fatty acids including the capric acid, stearic acid, lauric acid, palmitic acid and myristic acid and mixed them to prepare10 kinds of binary mixtures of fatty acids according to the predetermined proportion,tested the phase change temperature and latent heat of mixtures by differential scanning calorimetry(DSC). In order to find the fatty acid mixture which has suitable phase change temperature, the larger phase change latent heat and can be used for phase change wall. The results showed that the phase change temperature and latent heats of the binary mixtures of fatty acids decreased compared with the single component;The phase change temperature of the binary mixtures of fatty acids containing capric acid were lower, the range was roughly 20∼30°C,and latent heat is large,which are ideal phase change materials for phase change wall energy storage;The phase change temperature of the binary mixtures consisting of other fatty acids were still high,didn’t meet the temperature requirements of the wall energy storage.

Fatty acid profile, color and lipid oxidation of organic fermented sausage during chilling storage as influenced by acid whey and probiotic strains addition

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Karolina Maria Wójciak

2015-02-01

Full Text Available Organic fermented sausages typically spoil during long-term storage due to oxidative rancidity. The application of natural antioxidants to meat stuffing is a major practice intended to inhibit the oxidation process and color changes. This study aimed to assess the effect of two unusual starter cultures: three probiotic strains (Lactobacillus casei LOCK 0900, Lactobacillus casei LOCK 0908 and Lactobacillus paracasei LOCK 0919 and lactic acid bacteria from acid whey on model fermented sausage type products focusing on oxidative stability by measuring instrumental color (L*, a*, b* values, conjugated dienes (CD, TBARS immediately after 21 days of ripening (0 and after 90 and 180 days of refrigerated storage (4 ºC. Determination of fatty acid composition, in meat product was performed after ripening and after 180 days of storage. At the end of the storage period, the salted sausages were characterized by the same content of polyunsaturated fatty acids (PUFA compared to cured samples. The addition of acid whey and a mixture of probiotic strains to nitrite-free sausage formulation was barely able to protect lipids against oxidation in comparison to nitrite during vacuum storage. Surprisingly, the use of acid whey has an influence on the desired red-pinkish color of organic fermented sausage after ripening and after 180 days of storage period.

Free polyunsaturated fatty acids cause taste deterioration of salmon during frozen storage

DEFF Research Database (Denmark)

Refsgaard, Hanne; Brockhoff, P.M.B.; Jensen, Benny

2000-01-01

Increased intensity of train oil taste, bitterness, and metal taste are the most pronounced sensory changes during frozen storage of salmon (Refsgaard, H. H. F.; Brockhoff, P. B.; Jensen, B. Sensory and Chemical Changes in Farmed Atlantic Salmon (Salmo salar) during Frozen Storage. J. Agric. Food...... Chem. 1998a, 46, 3473-3479). Addition of each of the unsaturated fatty acids: palmitoleic acid (16:1, n - 7), linoleic acid (C18:2, it - 6), eicosapentaenoic acid (EPA; C20:5, it - 3) and docosahexaenoic acid (DHA; C22:6, n. - 3) to fresh minced salmon changed the sensory perception and increased...... the intensity of train oil taste, bitterness, and metal taste. The added level of each fatty acid (similar to 1 mg/g salmon meat) was equivalent to the concentration of the fatty acids determined in salmon stored as fillet at -10 degrees C for 6 months. The effect of addition of the fatty acids on the intensity...

Lysosomal storage diseases and the blood-brain barrier.

Science.gov (United States)

Begley, David J; Pontikis, Charles C; Scarpa, Maurizio

2008-01-01

The blood-brain barrier becomes a crucial issue in neuronopathic lysosomal storage diseases for three reasons. Firstly, the function of the blood-brain barrier may be compromised in many of the lysosomal storage diseases and this barrier dysfunction may contribute to the neuropathology seen in the diseases and accelerate cell death. Secondly, the substrate reduction therapies, which successfully reduce peripheral lysosomal storage, because of the blood-brain barrier may not have as free an access to brain cells as they do to peripheral cells. And thirdly, enzyme replacement therapy appears to have little access to the central nervous system as the mannose and mannose-6-phosphate receptors involved in their cellular uptake and transport to the lysosome do not appear to be expressed at the adult blood-brain barrier. This review will discuss in detail these issues and their context in the development of new therapeutic strategies.

Effect of curd freezing and packaging methods on the organic acid contents of goat cheeses during storage

Directory of Open Access Journals (Sweden)

Pınar Balkir

2011-09-01

Full Text Available Effects of freezing and packaging methods on organic acid content of goat cheese during 12 weeks of storage were determined. Goat cheese milk curds were divided into two batches; one of the batches was directly processed in to goat cheese while the other was frozen at -18 °C and stored for six months and processed into cheese after being thawed. Cheese samples were packed in three parts and stored at 4 °C refrigerated control sample and at -18 °C for six months frozen experimental samples. Cheese samples were packed in three different packaging methods: aerobic, vacuum or modified atmosphere. Citric, malic, fumaric, acetic, lactic, pyruvic and propionic acids were analyzed using HPLC method after 1st, 3rd, 6th, 9th and 12th week of storage period. Lactic acid was the main organic acids while pyruvic acid had the lowest content in all cheese samples. Citric and fumaric acid levels of frozen samples increased during storage whereas malic, acetic, pyruvic and propionic acid amounts were decreased compared to the beginning of storage. Packaging methods and freezing process also effected lactic acid levels statistically (p<0.05. Fumaric, acetic and lactic acid concentration of refrigerated samples were increased but citric, malic and propionic acids decreased during storage. Pyruvic acid level did not change significantly. It was determined that organic acid concentrations were effected by freezing process, storage time and packaging methods significantly (p<0.05.

Effect of postharvest methyl jasmonate treatment on fatty acid composition and phenolic acid content in olive fruits during storage.

Science.gov (United States)

Flores, Gema; Blanch, Gracia Patricia; Del Castillo, María Luisa Ruiz

2017-07-01

The nutritional effects of both table olives and olive oil are attributed not only to their fatty acids but also to antioxidant phenolics such as phenolic acids. Delays in oil processing usually result in undesirable oxidation and hydrolysis processes leading to formation of free fatty acids. These alterations create the need to process oil immediately after olive harvest. However, phenolic content decreases drastically during olive storage resulting in lower quality oil. In the present study we propose postharvest methyl jasmonate treatment as a mean to avoid changes in fatty acid composition and losses of phenolic acids during olive storage. Contents of fatty acids and phenolic acids were estimated in methyl jasmonate treated olives throughout 30-day storage, as compared with those of untreated olives. Significant decreases of saturated fatty acids were observed in treated samples whereas increases of oleic, linoleic and linolenic acids were respectively measured (i.e. from 50.8% to 64.5%, from 7.2% to 9.1% and from 1.5% to 9.3%). Also, phenolic acid contents increased significantly in treated olives. Particularly, increases of gallic acid from 1.35 to 6.29 mg kg -1 , chlorogenic acid from 9.18 to 16.21 mg kg -1 , vanillic acid from 9.61 to 16.99 mg kg -1 , caffeic acid from 5.12 to 12.55 mg kg -1 , p-coumaric acid from 0.96 to 5.31 mg kg -1 and ferulic acid from 4.05 to 10.43 mg kg -1 were obtained. Methyl jasmonate treatment is proposed as an alternative postharvest technique to traditional methods to guarantee olive oil quality when oil processing is delayed and olive fruits have to necessarily to be stored. © 2016 Society of Chemical Industry. © 2016 Society of Chemical Industry.

Kefir Grains Change Fatty Acid Profile of Milk during Fermentation and Storage

Science.gov (United States)

Vieira, C. P.; Álvares, T. S.; Gomes, L. S.; Torres, A. G.; Paschoalin, V. M. F.; Conte-Junior, C. A.

2015-01-01

Several studies have reported that lactic acid bacteria may increase the production of free fatty acids by lipolysis of milk fat, though no studies have been found in the literature showing the effect of kefir grains on the composition of fatty acids in milk. In this study the influence of kefir grains from different origins [Rio de Janeiro (AR), Viçosa (AV) e Lavras (AD)], different time of storage, and different fat content on the fatty acid content of cow milk after fermentation was investigated. Fatty acid composition was determined by gas chromatography. Values were considered significantly different when pkefir relatively to fermented kefir leading to possible increase of antimutagenic and anticarcinogenic potential and improvement of nutritional quality of lipids in storage milk. Only high-lipidic matrix displayed increase polyunsaturated fatty acids after fermentation. These findings open up new areas of study related to optimizing desaturase activity during fermentation in order to obtaining a fermented product with higher nutritional lipid quality. PMID:26444286

Domoic Acid Epileptic Disease

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John S. Ramsdell

2014-03-01

Full Text Available Domoic acid epileptic disease is characterized by spontaneous recurrent seizures weeks to months after domoic acid exposure. The potential for this disease was first recognized in a human case study of temporal lobe epilepsy after the 1987 amnesic shellfish-poisoning event in Quebec, and was characterized as a chronic epileptic syndrome in California sea lions through investigation of a series of domoic acid poisoning cases between 1998 and 2006. The sea lion study provided a breadth of insight into clinical presentations, unusual behaviors, brain pathology, and epidemiology. A rat model that replicates key observations of the chronic epileptic syndrome in sea lions has been applied to identify the progression of the epileptic disease state, its relationship to behavioral manifestations, and to define the neural systems involved in these behavioral disorders. Here, we present the concept of domoic acid epileptic disease as a delayed manifestation of domoic acid poisoning and review the state of knowledge for this disease state in affected humans and sea lions. We discuss causative mechanisms and neural underpinnings of disease maturation revealed by the rat model to present the concept for olfactory origin of an epileptic disease; triggered in dendodendritic synapases of the olfactory bulb and maturing in the olfactory cortex. We conclude with updated information on populations at risk, medical diagnosis, treatment, and prognosis.

Lysosomes, Lysosomal Storage Diseases, and Inflammation

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Calogera M. Simonaro PhD

2016-05-01

Full Text Available Lysosomes were originally described in the early 1950s by de Duve who was also the first to recognize the importance of these organelles in human disease. We know now that lysosomes are involved in numerous biological processes, and abnormalities in lysosomal function may result in a broad range of diseases. This review will briefly discuss the role of lysosomes in inflammation and how disruption of normal lysosomal function in the lysosomal storage diseases (LSDs leads to abnormalities in inflammation and immunity.

Neutropenia, neutrophil dysfunction, and inflammatory bowel disease in glycogen storage disease type Ib : Results of the European Study on Glycogen Storage Disease Type I

NARCIS (Netherlands)

Visser, G; Rake, JP; Fernandes, J; Labrune, P; Leonard, JV; Moses, S; Ullrich, K; Smit, GPA

Objective: To investigate the incidence, the severity, and the course of neutropenia, neutrophil dysfunction, and inflammatory bowel disease (IBD) in glycogen storage disease (GSD) type Ib. Method: As part of a collaborative European Study on GSD type I, a retrospective registry was established in

DYNAMICS AND INTENSITY OF APLE DISEASE DEVELOPMENT DURING ITS STORAGE

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D. Ivić

2006-12-01

Full Text Available Post-harvest apple fruit diseases are primarily caused by fungi. The object of this research was to quantify yield loss caused by post-harvest diseases and to determine fungal species responsible for storage rots on the cv. Idared during three months of storage, as well as to compare the development of fungi inoculated on apple fruits in the laboratory conditions. Only fruits with the visible rot symptoms were regarded as "diseased". Total yield loss during all three months of storage was 1.9%. The percentage of diseased fruits increased from the harvest moment to the end of storage. The most frequent cause of post-harvest rot in all assessments was Monilia fructigena. Penicillium species and Botrytis cinerea were present in relatively high percentage. All fungal isolates from diseased fruits caused fruit rot when inoculated on apples and incubated for 28 days at 22°C. On fruits inoculated with the same isolates and incubated at 4°C rot development was slower. Isolates of M. fructigena developed most rapidly on inoculated fruits at 22°C, while the isolates of B. cinerea developed most rapidly at 4°C.

Retention of short chain fatty acids under drying and storage conditions

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Alexandre Santos Souza

2011-09-01

Full Text Available Cheese whey permeate was used as a substrate for the fermentation of Propionibacterium freudenreichi PS1 for the production of short chain fatty acids, components of the bio-aroma of Swiss cheese. The liquid bio-aroma was encapsulated by spray drying under different conditions of air inlet temperature and feed rate. A study was carried out on the stability of the bio-aroma during storage in laminated packages at 30 °C for 96 days using the product showing the greatest retention of acetic and propionic acids. The results showed that the best drying conditions were an air entrance temperature of 180 °C and a feed rate of 24 g/min resulting in particles with a smooth surface and few invaginations and micro-fissures. However, 72% of the acetic acid and 80% of the propionic acid were lost during storage showing that the wall material used was inadequate to guarantee product stability.

Glycogen storage disease type Ia: linkage of glucose, glycogen, lactic acid, triglyceride, and uric acid metabolism.

Science.gov (United States)

Sever, Sakine; Weinstein, David A; Wolfsdorf, Joseph I; Gedik, Reyhan; Schaefer, Ernst J

2012-01-01

A female presented in infancy with hypotonia, undetectable serum glucose, lactic acidosis, and triglycerides >5000 mg/dL. The diagnosis of type 1A glycogen storage disease was made via the result of a liver biopsy, which showed increased glycogen and absent glucose-6-phosphatase enzyme activity. The patient was treated with dextrose administered orally, which was replaced by frequent feedings of cornstarch, which resulted in an improvement of her metabolic parameters. At age 18 years of age, she had marked hypertriglyceridemia (3860 mg/dL) and eruptive xanthomas and was treated with fenofibrate, atorvastatin, and fish oil. At age 29 years she was noted to have multiple liver adenomas, severe anemia, and hyperuricemia. Aggressive cornstarch therapy was commenced with a goal of maintaining her blood glucose levels >75 mg/dL and lactate levels triglycerides 179, high-density lipoprotein cholesterol 32, and calculated low-density lipoprotein cholesterol 154. Her weight was stable with a body mass index of 24.8 kg/m(2). Her liver adenomas had decreased in size, and her anemia and hyperuricemia had improved. She was homozygous for the R83C missense mutation in G6PC. Our data indicate that optimized metabolic control to maintain blood glucose levels >75 mg/dL is critical in the management of this disease. Copyright © 2012. Published by Elsevier Inc.

Basic and acidic leaching of Melton Valley Storage Tank sludge at Oak Ridge National Laboratory

International Nuclear Information System (INIS)

Collins, J.L.; Egan, B.Z.; Beahm, E.C.

1995-01-01

Basic and acidic leaching tests were conducted with samples of sludge taken from an underground storage tank at the US Department of Energy Melton Valley Storage Tank facility at Oak Ridge National Laboratory. The tests evaluated separation technologies for use in sludge processing to concentrate the radionuclides and reduce the volumes of storage tank waste for final disposal. Study results of sludge characterization, caustic leaching of sludge samples at ambient temperature and at 95 degrees C, and acid leaching of sludge samples at ambient temperature are reported in detail

Skeletal muscle metabolism is impaired during exercise in glycogen storage disease type III

DEFF Research Database (Denmark)

Preisler, Nicolai; Laforêt, Pascal; Madsen, Karen Lindhardt

2015-01-01

/kg/min (p = 0.024). Fructose ingestion improved exercise tolerance in the patients. CONCLUSION: Similar to patients with McArdle disease, in whom muscle glycogenolysis is also impaired, GSDIIIa is associated with a reduced skeletal muscle oxidation of carbohydrates and a compensatory increase in fatty acid......OBJECTIVE: Glycogen storage disease type IIIa (GSDIIIa) is classically regarded as a glycogenosis with fixed weakness, but we hypothesized that exercise intolerance in GSDIIIa is related to muscle energy failure and that oral fructose ingestion could improve exercise tolerance in this metabolic...... myopathy. METHODS: We challenged metabolism with cycle-ergometer exercise and measured substrate turnover and oxidation rates using stable isotope methodology and indirect calorimetry in 3 patients and 6 age-matched controls on 1 day, and examined the effect of fructose ingestion on exercise tolerance...

Liver and Skin Histopathology in Adults with Acid Sphingomyelinase Deficiency (Niemann-Pick Disease Type B)

Science.gov (United States)

Thurberg, Beth L.; Wasserstein, Melissa P.; Schiano, Thomas; O’Brien, Fanny; Richards, Susan; Cox, Gerald F.; McGovern, Margaret M.

2012-01-01

Acid sphingomyelinase deficiency (ASMD) is a lysosomal storage disorder characterized by the pathologic accumulation of sphingomyelin in multiple cells types, and occurs most prominently within the liver, spleen and lungs, leading to significant clinical disease. Seventeen ASMD patients underwent a liver biopsy during baseline screening for a Phase 1 trial of recombinant human acid sphingomyelinase (rhASM) in adults with Niemann-Pick disease type B. Eleven of the 17 were enrolled in the trial and each received a single dose of rhASM and underwent a repeat liver biopsy on Day 14. Biopsies were evaluated for fibrosis, sphingomyelin accumulation and macrophage infiltration by light and electron microscopy. When present, fibrosis was periportal and pericellular, predominantly surrounding affected Kupffer cells. Two baseline biopsies exhibited frank cirrhosis. Sphingomyelin was localized to isolated Kupffer cells in mildly affected biopsies and was present in both Kupffer cells and hepatocytes in more severely affected cases. Morphometric quantification of sphingomyelin storage in liver biopsies ranged from 4–44% of the microscopic field. Skin biopsies were also performed at baseline and Day 14 in order to compare the sphingomyelin distribution in a peripheral tissue to that of liver. Sphingomyelin storage was present at lower levels in multiple cell types of the skin, including dermal fibroblasts, macrophages, vascular endothelial cells, vascular smooth muscle cells and Schwann cells. This Phase 1 trial of rhASM in adults with ASMD provided a unique opportunity for a prospective assessment of hepatic and skin pathology in this rare disease and their potential usage as pharmacodynamic biomarkers. PMID:22613999

78 FR 58574 - Maintenance, Testing, and Replacement of Vented Lead-Acid Storage Batteries for Nuclear Power Plants

Science.gov (United States)

2013-09-24

...-Acid Storage Batteries for Nuclear Power Plants AGENCY: Nuclear Regulatory Commission. ACTION... for Nuclear Power Plants.'' The guide describes methods that the NRC staff considers acceptable for... replacement of vented lead-acid storage batteries in nuclear power plants. ADDRESSES: Please refer to Docket...

Case Report: Ursodeoxycholic acid treatment in Niemann-Pick disease type C; clinical experience in four cases

Science.gov (United States)

Movsesyan, Nina; Platt, Frances M.

2017-01-01

In this case series, we demonstrate that Ursodeoxycholic acid (UDCA) improves liver dysfunction in Niemann-Pick type C (NPC) and may restore a suppressed cytochrome p450 system. NPC disease is a progressive neurodegenerative lysosomal storage disease caused by mutations in either the NPC1 or NPC2 genes. Liver disease is a common feature presenting either acutely as cholestatic jaundice in the neonatal period, or in later life as elevated liver enzymes indicative of liver dysfunction. Recently, an imbalance in bile acid synthesis in a mouse model of NPC disease was linked to suppression of the P450 detoxification system and was corrected by UDCA treatment. UDCA (3α, 7β-dihydroxy-5β-cholanic acid), a hydrophilic bile acid, is used to treat various cholestatic disorders. In this report we summarise the findings from four independent cases of NPC, three with abnormal liver enzyme levels at baseline, that were subsequently treated with UDCA. The patients differed in age and clinical features, they all tolerated the drug well, and in those with abnormal liver function, there were significant improvements in their liver enzyme parameters. PMID:29119141

Effect of Storage Temperature on Vitamin C, Total Phenolics, UPLC Phenolic Acid Profile and Antioxidant Capacity of Eleven Potato (Solanum tuberosum Varieties

Directory of Open Access Journals (Sweden)

Joseph Hubert Yamdeu Galani

2017-03-01

Full Text Available Storage of potato tubers at low temperature affects their metabolism and may alter their phytochemical properties. There is a need to elucidate the changes in antioxidant compounds, activity and enzymes during storage of tubers. Eleven Indian potato varieties were evaluated for antioxidant parameters, after 0, 30, 60 and 90 days of storage at room temperature, 15 °C and 4 °C. Total phenolics (0.0786–0.1546 mg gallic acid equivalents⋅g−1 FW and vitamin C content (0.0828–0.2416 mg⋅g−1 FW varied among the varieties and were different with storage temperature; their levels fluctuated during storage but remained above the initial level until the last day of observation. Phenolic acid profiling by UPLC identified 12 compounds among which the most abundant was chlorogenic acid followed by gallic acid, sinapic acid and ellagic acid. Except para-coumaric acid which decreased at 4 °C, all the phenolic acids increased with storage. Caffeic acid, chlorogenic acid, protocatechuic acid and gallic acid mostly correlated with total phenolic content (r = 0.456, 0.482, 0.588 and 0.620, respectively. Antioxidant activity against both DPPH and ABTS radicals increased during the initial days of storage and then dropped to a level comparable or lower than the original value, irrespective of the storage temperature. Correlation study revealed that chlorogenic acid, gallic acid and ferulic acid mostly contributed to antioxidant activity. Activity of both antioxidant enzymes, superoxide dismutase and ascorbate peroxidase, increased initially but then decreased to values lower than the initial level and were not influenced by storage temperature. Correlation with antioxidant activity indicated that the enhancement of reactive oxygen scavenging species in cold stored tubers could result mainly from ascorbate peroxidase activity. Our results demonstrate that storage temperature adversely influences the metabolism and the content of

Use of complementary and alternative medicine by patients with lysosomal storage diseases.

Science.gov (United States)

Balwani, Manisha; Fuerstman, Laura; Desnick, Robert J; Buckley, Brian; McGovern, Margaret M

2009-10-01

To evaluate the extent of complementary and alternative medicine use and perceived effectiveness in patients with lysosomal storage diseases. A 26-item survey was distributed to 495 patients with type 1 Gaucher, Fabry, and type B Niemann-Pick diseases who were seen at the Lysosomal Storage Disease Program at the Mount Sinai School of Medicine. Survey responses were entered into an access database and analyzed using descriptive statistics. Surveys were completed by 167 respondents with an overall response rate of 34%. Complementary and alternative medicines were used by 45% of patients with type 1 Gaucher disease, 41% of patients with Fabry disease, and 47% of patients with type B Niemann-Pick for symptoms related to their disease. Complementary and alternative medicines were used most frequently by adult females (55%), in patients who reported having one or more invasive procedures due to their disease, patients who use one or more conventional medical therapies, or those with depression and/or anxiety. Overall perceived effectiveness of complementary and alternative medicine supplements was low; however, complementary and alternative medicine therapies were perceived as effective. Complementary and alternative medicines are commonly used among patients with lysosomal storage diseases. Assessment of the effectiveness of these approaches in the lysosomal storage diseases is needed, and physicians should be aware of complementary and alternative medicine therapies used by patients to evaluate safety and possible drug interactions.

Kefir Grains Change Fatty Acid Profile of Milk during Fermentation and Storage.

Science.gov (United States)

Vieira, C P; Álvares, T S; Gomes, L S; Torres, A G; Paschoalin, V M F; Conte-Junior, C A

2015-01-01

Several studies have reported that lactic acid bacteria may increase the production of free fatty acids by lipolysis of milk fat, though no studies have been found in the literature showing the effect of kefir grains on the composition of fatty acids in milk. In this study the influence of kefir grains from different origins [Rio de Janeiro (AR), Viçosa (AV) e Lavras (AD)], different time of storage, and different fat content on the fatty acid content of cow milk after fermentation was investigated. Fatty acid composition was determined by gas chromatography. Values were considered significantly different when p<0.05. The highest palmitic acid content, which is antimutagenic compost, was seen in AV grain (36.6g/100g fatty acids), which may have contributed to increasing the antimutagenic potential in fermented milk. Higher monounsaturated fatty acid (25.8 g/100g fatty acids) and lower saturated fatty acid (72.7 g/100g fatty acids) contents were observed in AV, when compared to other grains, due to higher Δ9-desaturase activity (0.31) that improves the nutritional quality of lipids. Higher oleic acid (25.0 g/100g fatty acids) and monounsaturated fatty acid (28.2g/100g fatty acids) and lower saturated fatty acid (67.2g/100g fatty acids) contents were found in stored kefir relatively to fermented kefir leading to possible increase of antimutagenic and anticarcinogenic potential and improvement of nutritional quality of lipids in storage milk. Only high-lipidic matrix displayed increase polyunsaturated fatty acids after fermentation. These findings open up new areas of study related to optimizing desaturase activity during fermentation in order to obtaining a fermented product with higher nutritional lipid quality.

Pregnancies in glycogen storage disease type Ia

NARCIS (Netherlands)

Martens, Danielle H. J.; Rake, Jan Peter; Schwarz, Martin; Ullrich, Kurt; Weinstein, David A.; Merkel, Martin; Sauer, Pieter J. J.; Smit, G. Peter A.

OBJECTIVE: Reports on pregnancies in women with glycogen storage disease type Ia (GSD-Ia) are scarce. Because of improved life expectancy, pregnancy is becoming an important issue. We describe 15 pregnancies by focusing on dietary treatment, biochemical parameters, and GSD-Ia complications. STUDY

Granulocyte colony-stimulating factor in glycogen storage disease type 1b. Results of the European Study on Glycogen Storage Disease Type 1

NARCIS (Netherlands)

Visser, G.; Rake, J.P.; Labrune, P.; Leonard, J.V.; Moses, S.; Ullrich, K.; Wendel, U.; Groenier, K.H.; Smit, G.P.

2002-01-01

Patients with glycogen storage disease type 1b (GSD-1b) have neutropenia and neutrophil dysfunction that predispose to frequent infections and inflammatory bowel disease (IBD), for which granulocyte colony-stimulating factor (GCSF) is given. To investigate the use and the value of GCSF treatment in

Analysis of lipid profile in lipid storage myopathy.

Science.gov (United States)

Aguennouz, M'hammed; Beccaria, Marco; Purcaro, Giorgia; Oteri, Marianna; Micalizzi, Giuseppe; Musumesci, Olimpia; Ciranni, Annmaria; Di Giorgio, Rosa Maria; Toscano, Antonio; Dugo, Paola; Mondello, Luigi

2016-09-01

Lipid dysmetabolism disease is a condition in which lipids are stored abnormally in organs and tissues throughout the body, causing muscle weakness (myopathy). Usually, the diagnosis of this disease and its characterization goes through dosage of Acyl CoA in plasma accompanied with evidence of droplets of intra-fibrils lipids in the patient muscle biopsy. However, to understand the pathophysiological mechanisms of lipid storage diseases, it is useful to identify the nature of lipids deposited in muscle fiber. In this work fatty acids and triglycerides profile of lipid accumulated in the muscle of people suffering from myopathies syndromes was characterized. In particular, the analyses were carried out on the muscle biopsy of people afflicted by lipid storage myopathy, such as multiple acyl-coenzyme A dehydrogenase deficiency, and neutral lipid storage disease with myopathy, and by the intramitochondrial lipid storage dysfunctions, such as deficiencies of carnitine palmitoyltransferase II enzyme. A single step extraction and derivatization procedure was applied to analyze fatty acids from muscle tissues by gas chromatography with a flame ionization detector and with an electronic impact mass spectrometer. Triglycerides, extracted by using n-hexane, were analyzed by high performance liquid chromatography coupled to mass spectrometer equipped with an atmospheric pressure chemical ionization interface. The most representative fatty acids in all samples were: C16:0 in the 13-24% range, C18:1n9 in the 20-52% range, and C18:2n6 in the 10-25% range. These fatty acids were part of the most representative triglycerides in all samples. The data obtained was statistically elaborated performing a principal component analysis. A satisfactory discrimination was obtained among the different diseases. Using component 1 vs component 3 a 43.3% of total variance was explained. Such results suggest the important role that lipid profile characterization can have in supporting a correct

Effects of storage conditions on the fatty acid composition of the ...

African Journals Online (AJOL)

SARAH

2015-08-31

Aug 31, 2015 ... Key words: Pentadesma butyracea, oil seed, storage, butter, fatty acid. INTRODUCTION ... oil, in skin and hair care and in the manufacturing of soap because of its ..... study of Ozonized olive oil and ozonized sunflower oil.

Blocked muscle fat oxidation during exercise in neutral lipid storage disease

DEFF Research Database (Denmark)

Laforêt, Pascal; Ørngreen, Mette; Preisler, Nicolai

2012-01-01

To determine whether impaired exercise capacity in neutral lipid storage disease with myopathy is solely caused by muscle weakness or whether a defect in energy metabolism (blocked fat oxidation) may also play a role.......To determine whether impaired exercise capacity in neutral lipid storage disease with myopathy is solely caused by muscle weakness or whether a defect in energy metabolism (blocked fat oxidation) may also play a role....

Tay Sachs and Related Storage Diseases: Family Planning

Science.gov (United States)

Schneiderman, Gerald; And Others

1978-01-01

Based on interviews with 24 families, the article discusses family planning and the choices available to those families in which a child has previously died from Tay-Sachs or related lipid storage diseases. (IM)

The lysosomal storage disease continuum with ageing-related neurodegenerative disease.

Science.gov (United States)

Lloyd-Evans, Emyr; Haslett, Luke J

2016-12-01

Lysosomal storage diseases and diseases of ageing share many features both at the physiological level and with respect to the mechanisms that underlie disease pathogenesis. Although the exact pathophysiology is not exactly the same, it is astounding how many similar pathways are altered in all of these diseases. The aim of this review is to provide a summary of the shared disease mechanisms, outlining the similarities and differences and how genetics, insight into rare diseases and functional research has changed our perspective on the causes underlying common diseases of ageing. The lysosome should no longer be considered as just the stomach of the cell or as a suicide bag, it has an emerging role in cellular signalling, nutrient sensing and recycling. The lysosome is of fundamental importance in the pathophysiology of diseases of ageing and by comparing against the LSDs we not only identify common pathways but also therapeutic targets so that ultimately more effective treatments can be developed for all neurodegenerative diseases. Copyright © 2016. Published by Elsevier B.V.

Rheology, fatty acid profile and storage characteristics of cookies as influenced by flax seed (Linum usitatissimum).

Science.gov (United States)

Rajiv, Jyotsna; Indrani, Dasappa; Prabhasankar, Pichan; Rao, G Venkateswara

2012-10-01

Flaxseed is a versatile functional ingredient owing to its unique nutrient profile. Studies on the effect of substitution of roasted and ground flaxseed (RGF) at 5, 10, 15 and 20% level on the wheat flour dough properties showed that amylograph peak viscosity, farinograph dough stability, extensograph resistance to extension and extensibility values decreased with the increase in the substitution of RGF from 0-20%. The cookie baking test showed a marginal decrease in spread ratio but beyond substitution of 15% RGF the texture and flavour of the cookies was adversely affected. The data on storage characteristics of control and cookies with 15% RGF showed no significant change with respect to acidity of extracted fat and peroxide values due to storage of cookies upto 90 days in metallised polyester pouches at ambient conditions. The gas chromatographic analysis of fatty acid profile indicated that the control cookies contained negligible linolenic acid and the flaxseed cookies contained 4.75 to 5.31% of linolenic acid which showed a marginal decrease over storage. Hence flaxseed could be used as a source of omega-3-fatty acid.

Method of quantifying the loss of acidification activity of lactic acid starters during freezing and frozen storage.

Science.gov (United States)

Fonseca, F; Béal, C; Corrieu, G

2000-02-01

We have developed a method to quantify the resistance to freezing and frozen storage of lactic acid starters, based on measuring the time necessary to reach the maximum acidification rate in milk (tm) using the Cinac system. Depending on the operating conditions, tm increased during the freezing step and storage. The loss of acidification activity during freezing was quantified by the difference (delta tm) between the tm values of the concentrated cell suspension before and after freezing. During storage at -20 degrees C, linear relationships between tm and the storage time were established. Their slope, k, allowed the quantitation of the decrease in acidification activity during 9-14 weeks of frozen storage. The method was applied to determine the resistance to freezing and frozen storage of four strains of lactic acid bacteria and to quantify the cryoprotective effect of glycerol.

Effect of storage temperature in a Cambodian field setting on the fatty acid composition in whole blood

DEFF Research Database (Denmark)

Nurhasan, Mulia; Roos, Nanna; Aristizabal Henao, J J

2015-01-01

and -80°C samples did not differ, but there was relatively more highly unsaturated fatty acids (15.8±2.7 vs. 14.4±2.5%, p4, p...Fatty acid analysis requires standardized collection and storage of samples, which can be a challenge under field conditions. This study describes the effect of storage temperature on fatty acid composition in two sets of whole blood samples collected from 66 children in a rural area in Cambodia...

Consensus guidelines for management of glycogen storage disease type 1b - European Study on Glycogen Storage Disease Type 1

NARCIS (Netherlands)

Visser, G; Rake, JP; Labrune, P; Leonard, JV; Moses, S; Ullrich, K; Wendel, U; Smit, GPA

2002-01-01

Life expectancy in glycogen storage disease type 1 (GSD-1) has improved considerably. Its relative rarity implies that no metabolic centre has experience of large series of patients and therefore experience with long-term management and follow-up at each centre is limited. There is wide variation in

Guidelines for management of glycogen storage disease type I - European study on glycogen storage disease type I (ESGSD I)

NARCIS (Netherlands)

Rake, JP; Visser, G; Labrune, P; Leonard, JV; Ullrich, K; Smit, GPA

2002-01-01

Life-expectancy in glycogen storage disease type I (GSD I) has improved considerably. Its relative rarity implies that no metabolic centre has experience of large series of patients and experience with long-term management and follow-up at each centre is limited. There is wide variation in methods

Chronic cyclodextrin treatment of murine Niemann-Pick C disease ameliorates neuronal cholesterol and glycosphingolipid storage and disease progression.

Directory of Open Access Journals (Sweden)

Cristin D Davidson

2009-09-01

Full Text Available Niemann-Pick type C (NPC disease is a fatal neurodegenerative disorder caused most commonly by a defect in the NPC1 protein and characterized by widespread intracellular accumulation of unesterified cholesterol and glycosphingolipids (GSLs. While current treatment therapies are limited, a few drugs tested in Npc1(-/- mice have shown partial benefit. During a combination treatment trial using two such compounds, N-butyldeoxynojirimycin (NB-DNJ and allopregnanolone, we noted increased lifespan for Npc1(-/- mice receiving only 2-hydroxypropyl-beta-cyclodextrin (CD, the vehicle for allopregnanolone. This finding suggested that administration of CD alone, but with greater frequency, might provide additional benefit.Administration of CD to Npc1(-/- mice beginning at either P7 or P21 and continuing every other day delayed clinical onset, reduced intraneuronal cholesterol and GSL storage as well as free sphingosine accumulation, reduced markers of neurodegeneration, and led to longer survival than any previous treatment regime. We reasoned that other lysosomal diseases characterized by cholesterol and GSL accumulation, including NPC disease due to NPC2 deficiency, GM1 gangliosidosis and mucopolysaccharidosis (MPS type IIIA, might likewise benefit from CD treatment. Treated Npc2(-/- mice showed benefits similar to NPC1 disease, however, mice with GM1 gangliosidosis or MPS IIIA failed to show reduction in storage.Treatment with CD delayed clinical disease onset, reduced intraneuronal storage and secondary markers of neurodegeneration, and significantly increased lifespan of both Npc1(-/- and Npc2(-/- mice. In contrast, CD failed to ameliorate cholesterol or glycosphingolipid storage in GM1 gangliosidosis and MPS IIIA disease. Understanding the mechanism(s by which CD leads to reduced neuronal storage may provide important new opportunities for treatment of NPC and related neurodegenerative diseases characterized by cholesterol dyshomeostasis.

Excessive burden of lysosomal storage disorder gene variants in Parkinson's disease

NARCIS (Netherlands)

Robak, L.A.; Jansen, I.E.; Rooij, J van; Uitterlinden, A.G.; Kraaij, R.; Jankovic, J.; Heutink, P.; Shulman, J.M.; Bloem, B.; Post, B.; Scheffer, H.; Warrenburg, B.P.C. van de; et al.,

2017-01-01

Mutations in the glucocerebrosidase gene (GBA), which cause Gaucher disease, are also potent risk factors for Parkinson's disease. We examined whether a genetic burden of variants in other lysosomal storage disorder genes is more broadly associated with Parkinson's disease susceptibility. The

Transformation of chenodeoxycholic acid to ursodeoxycholic acid in patients with Crohn's disease

International Nuclear Information System (INIS)

Miwa, H.; Yamamoto, M.; Nishida, T.; Yao, T.

1986-01-01

In vivo 7 beta-epimerization of chenodeoxycholic acid to ursodeoxycholic acid and the role of 7-ketolithocholic acid as an intermediate in this biotransformation were studied in 11 patients with Crohn's disease and in 5 healthy volunteers. The incorporation of deuterium into biliary ursodeoxycholic acid and 7-ketolithocholic acid was determined by computed gas chromatography-mass fragmentography after ingestion of a dideuterated chenodeoxycholic acid, chenodeoxycholic-11,12-d2 acid. The incorporation of deuterium into ursodeoxycholic acid increased to a peak level at 48 h in the patients with Crohn's disease, but was delayed in healthy volunteers. In 8 patients and 2 healthy controls there were small amounts of 7-ketolithocholic acid in bile. The incorporation of deuterium into 7-ketolithocholic acid was confirmed in only 2 patients and the peak level was noted at 48 h. These observations suggest that 7-ketolithocholic acid is an intermediate of this biotransformation in patients with Crohn's disease

Uric Acid, Hyperuricemia and Vascular Diseases

Science.gov (United States)

Jin, Ming; Yang, Fan; Yang, Irene; Yin, Ying; Luo, Jin Jun; Wang, Hong; Yang, Xiao-Feng

2011-01-01

Uric acid is the product of purine metabolism. It is known that hyperuricemia, defined as high levels of blood uric acid, is the major etiological factor of gout. A number of epidemiological reports have increasingly linked hyperuricemia with cardiovascular and neurological diseases. Studies highlighting the pathogenic mechanisms of uric acid point to an inflammatory response as the primary mechanism for inducing gout and possibly contributing to uric acid's vascular effects. Monosodium urate (MSU) crystals induce an inflammatory reaction, which are recognized by Toll-like receptors (TLRs). These TLRs then activate NALP3 inflammasome. MSU also triggers neutrophil activation and further produces immune mediators, which lead to a proinflammatory response. In addition, soluble uric acid can also mediate the generation of free radicals and function as a pro-oxidant. This review summarizes the epidemiological studies of hyperuricemia and cardiovascular disease, takes a brief look at hyperuricemia and its role in neurological diseases, and highlights the studies of the advanced pathological mechanisms of uric acid and inflammation. PMID:22201767

The capric and lauric acid mixture with chemical additives as latent heat storage materials for cooling application

Energy Technology Data Exchange (ETDEWEB)

Roxas-Dimaano, M.N. [University of Santo Tomas, Manila (Philippines). Research Center for the Natural Sciences; Watanabe, T. [Tokyo Institute of Technology (Japan). Research Laboratory for Nuclear Reactors

2002-09-01

The mixture of capric acid and lauric acid (C-L acid), with the respective mole composition of 65% and 35%, is a potential phase change material (PCM). Its melting point of 18.0{sup o}C, however, is considered high for cooling application of thermal energy storage. The thermophysical and heat transfer characteristics of the C-L acid with some organic additives are investigated. Compatibility of C-L acid combinations with additives in different proportions and their melting characteristics are analyzed using the differential scanning calorimeter (DSC). Among the chemical additives, methyl salicylate, eugenol, and cineole presented the relevant melting characteristics. The individual heat transfer behavior and thermal storage performance of 0.1 mole fraction of these additives in the C-L acid mixture are evaluated. The radial and axial temperature distribution during charging and discharging at different concentrations of selected PCM combinations are experimentally determined employing a vertical cylindrical shell and tube heat exchanger. The methyl salicylate in the C-L acid provided the most effective additive in the C-L acid. It demonstrated the least melting band width aimed at lowering the melting point of the C-L acid with the highest heat of fusion value with relatively comparable rate of heat transfer. Furthermore, the thermal performance based on the total amount of transferred energy and their rates, established the PCM's latent heat storage capability. (author)

The Effect of Storage Time, Temperature and Type of Packaging on the Release of Phthalate Esters into Packed Acidic Liquids

Directory of Open Access Journals (Sweden)

Noushin Rastkari

2017-01-01

Full Text Available Acidic liquids such as verjuice, lemon juice and vinegar are frequently consumed in Iran. Different kinds of acidic liquids are packaged in polyethylene terephthalate (PET and high-density polyethylene (HDPE bottles. There is evidence indicating that phthalates can leach from PET and HDPE bottles into their contents. In this work the effect of storage time, temperature and bottle type on the migration of phthalates from packaging materials into acidic liquids is studied by analyzing the samples stored under different conditions, before storage and after 2, 4 and 6 months of storage. The determined mean phthalate concentrations in μg/L were: <0.04 to 0.501 in verjuice, <0.04 to 0.231 in lemon juice and <0.04 to 0.586 in vinegar. The highest concentrations of diethyl phthalate (DEP and diethyl hexyl phthalate (DEHP were found in PET and HDPE bottles, respectively. Results of analyses before and after storage indicate that under some storage conditions, the concentrations of DEP, DEHP and dibutyl phthalate (DBP increased in acidic liquids. The possible migration of phthalic acid esters from plastic packaging materials into the contents was indicated by the results of the present study.

Lipids in hepatic glycogen storage diseases: pathophysiology, monitoring of dietary management and future directions.

Science.gov (United States)

Derks, Terry G J; van Rijn, Margreet

2015-05-01

Hepatic glycogen storage diseases (GSD) underscore the intimate relationship between carbohydrate and lipid metabolism. The hyperlipidemias in hepatic GSD reflect perturbed intracellular metabolism, providing biomarkers in blood to monitor dietary management. In different types of GSD, hyperlipidemias are of a different origin. Hypertriglyceridemia is most prominent in GSD type Ia and associated with long-term outcome morbidity, like pancreatitis and hepatic adenomas. In the ketotic subtypes of GSD, hypertriglyceridemia reflects the age-dependent fasting intolerance, secondary lipolysis and increased mitochondrial fatty acid oxidation. The role of high protein diets is established for ketotic types of GSD, but non-traditional dietary interventions (like medium-chain triglycerides and the ketogenic diet) in hepatic GSD are still controversial and necessitate further studies. Patients with these rare inherited disorders of carbohydrate metabolism meet several criteria of the metabolic syndrome, therefore close monitoring for cardiovascular diseases in ageing GSD patients may be justified.

Exercise intolerance in Glycogen Storage Disease Type III

DEFF Research Database (Denmark)

Preisler, Nicolai; Pradel, Agnès; Husu, Edith

2013-01-01

Myopathic symptoms in Glycogen Storage Disease Type IIIa (GSD IIIa) are generally ascribed to the muscle wasting that these patients suffer in adult life, but an inability to debranch glycogen likely also has an impact on muscle energy metabolism. We hypothesized that patients with GSD IIIa can...

Fluctuations in phenolic content, ascorbic acid and total carotenoids and antioxidant activity of fruit beverages during storage

Directory of Open Access Journals (Sweden)

C. Castro-López

2016-09-01

Full Text Available Stability of the total phenolic content, ascorbic acid, total carotenoids and antioxidant activity in eight fruit beverages was analyzed. The influence of storage temperature (4, 8 and 11 °C during the product shelf-life (20 days was evaluated. Pomegranate Juice presented the highest values for antioxidant activity by DPPH·− assay (552.93 ± 6.00 GAE μg mL−1, total carotenoids (3.18 ± 0.11 βCE μg mL−1, and total phenolic content (3967.07 ± 2.47 GAE μg mL−1; while Splash Blend recorded the highest levels of ascorbic acid (607.39 ± 2.13 AAE μg mL−1. The antioxidant capacity was stable at 4 and 8 °C for the first 8 days of storage; while carotenoids and ascorbic acid were slightly degraded through the storage time, possibly due to oxidation and/or reactions with other compounds. The results suggest that the observed variation during testing could be related to storage conditions of the final product.

Enhancement of heat transfer for thermal energy storage application using stearic acid nanocomposite with multi-walled carbon nanotubes

International Nuclear Information System (INIS)

Li, TingXian; Lee, Ju-Hyuk; Wang, RuZhu; Kang, Yong Tae

2013-01-01

A latent heat storage nanocomposite made of stearic acid (SA) and multi-walled carbon nanotube (MWCNT) is prepared for thermal energy storage application. The thermal properties of the SA/MWCNT nanocomposite are characterized by SEM (scanning electron microscopy) and DSC (differential scanning calorimeter) analysis techniques, and the effects of different volume fractions of MWCNT on the heat transfer enhancement and thermal performance of stearic acid are investigated during the charging and discharging phases. The SEM analysis shows that the additive of MWCNT is uniformly distributed in the phase change material of stearic acid, and the DSC analysis reveals that the melting point of SA/MWCNT nanocomposite shifts to a lower temperature during the charging phase and the freezing point shifts to a higher temperature during the discharging phase when compared with the pure stearic acid. The experimental results show that the addition of MWCNT can improve the thermal conductivity of stearic acid effectively, but it also weakens the natural convection of stearic acid in liquid state. In comparison with the pure stearic acid, the charging rate can be decreased by about 50% while the discharging rate can be improved by about 91% respectively by using the SA/5.0% MWCNT nanocomposite. It appears that the MWCNT is a promising candidate for enhancing the heat transfer performance of latent heat thermal energy storage system. - Highlights: • A nanocomposite made of stearic acid and multi-walled carbon nanotube is prepared for thermal energy storage application. • Effects of multi-walled carbon nanotube on the thermal performance of the nanocomposite are investigated. • Multi-walled carbon nanotube enhances the thermal conductivity but weakens the natural convection of stearic acid. • Discharging/charging rates of stearic acid are increased/decreased by using multi-walled carbon nanotube

Comparative effects of irradiation, fumigation, and storage on the free amino acids and sugar contents of green, black and oolong teas

International Nuclear Information System (INIS)

Kausar, Tusneem; Akram, Kashif; Kwon, Joong-Ho

2013-01-01

Food irradiation or chemical fumigation can be used to ensure the hygienic quality of teas. The comparative effects of gamma irradiation (5 and 10 kGy) and fumigation (MeBr and PH 3 ) were investigated on the amino acids and sugar contents of Camellia sinensis (green, black and oolong teas) during storage (15±12 °C). The major amino acids found in teas were theanine and glutamic acid. Irradiation increased amino acids such as, leucine, alanine, and glutamic acid, and decreased the histidine. PH 3 fumigation resulted in a decrease of tyrosine content; however, the effect of MeBr fumigation was negligible. Storage showed no significant effect on the amino acid content of the irradiated and fumigated teas. Sucrose, glucose, and fructose contents significantly increased upon gamma irradiation (p≤0.05). However, fumigation and subsequent storage did not affect the sugar contents. Irradiation could be a preferred alternative choice to address food safety problems as fumigation is restricted in many countries. - Highlights: ► Teas were investigated for the changes during storage on irradiation and fumigation. ► The effect on amino acid contents was more prominent on irradiation than fumigation. ► The sucrose, glucose, and fructose contents increased on gamma irradiation. ► Fumigation and storage did not affect the sugar contents in the teas. ► Irradiation could be an effective alternative for fumigants

Effect of harvest, drying and storage on the bitterness, moisture, sugars, free amino acids and phenolic compounds of jujube fruit (Zizyphus jujuba cv. Junzao).

Science.gov (United States)

Pu, Yunfeng; Ding, Tian; Wang, Wenjun; Xiang, Yanju; Ye, Xingqian; Li, Mei; Liu, Donghong

2018-01-01

The taste of dried jujube fruit when compared with fresh ones is less palatable, as it develops bitterness during drying and storage. Therefore, identifying the methods by which bitterness occurs is essential for developing strategies for processing and storage. Bitterness in fresh jujube fruit was negligible; however, it increased by 0.9-, 1.5- and 1.8-fold during drying and storage over 6 and 12 months. The moisture significantly decreased during harvesting and drying. Free amino acids, except proline and tyrosine, significantly decreased during drying and storage. Fructose, glucose and sucrose hardly changed during harvest, drying and storage. Titratable acidity, total phenolic and total flavonoids contents were stable during harvest and drying, but increased upon storage. Additionally, protocatechuic and ellagic acids were not detected in fresh jujube fruit, however, were found to increase during drying and storage. Bitterness in fresh jujube fruit tasted negligible because of meagre amount of phytochemicals, while the condensation effect of moisture reduction, the loss of free amino acids, and the formation of protocatechuic and ellagic acids could aggravate the bitterness of jujube fruit during drying and storage. © 2017 Society of Chemical Industry. © 2017 Society of Chemical Industry.

The Effect of Storage Time, Temperature and Type of Packaging on the Release of Phthalate Esters into Packed
Acidic Liquids.

Science.gov (United States)

Rastkari, Noushin; Zare Jeddi, Maryam; Yunesian, Masud; Ahmadkhaniha, Reza

2017-12-01

Acidic liquids such as verjuice, lemon juice and vinegar are frequently consumed in Iran. Different kinds of acidic liquids are packaged in polyethylene terephthalate (PET) and high-density polyethylene (HDPE) bottles. There is evidence indicating that phthalates can leach from PET and HDPE bottles into their contents. In this work the effect of storage time, temperature and bottle type on the migration of phthalates from packaging materials into acidic liquids is studied by analyzing the samples stored under different conditions, before storage and after 2, 4 and 6 months of storage. The determined mean phthalate concentrations in µg/L were: liquids. The possible migration of phthalic acid esters from plastic packaging materials into the contents was indicated by the results of the present study.

Effects of gamma irradiation and storage temperature on carotenoids and ascorbic acid content of mangoes on ripening

International Nuclear Information System (INIS)

Thomas, P.; Janave, M.T.

1975-01-01

Synthesis and accumulation of carotenoids in the flesh of Alphonso mangoes on ripening was found to be maximal in fruits stored at tropical ambient temperatures (28 0 to 32 0 C). Gamma irradiation of preclimacteric fruits at 25 krad did not affect the formation of carotenoids. Storage of preclimacteric fruits either irradiated or unirradiated at 7 to 20 0 C for 16 to 43 days caused a substantial reduction in carotenoid formation even when these fruits were subsequently ripened under optimal conditions. Regardless of storage temperature, carotenes always exceeded xanthophylls in the ripe fruits and, in general, irradiated fruits showed higher levels of carotenes in comparison with unirradiated samples. Ascorbic acid loss during ripening was maximum at ambient temperatures while lengthy storage at low temperatures caused a net increase in ascorbic acid levels. Irradiation seemed to accentuate the loss in ascorbic acid during ripening. (author)

Storage time of transfused blood and disease recurrence after colorectal cancer surgery

DEFF Research Database (Denmark)

Mynster, T; Nielsen, Hans Jørgen

2001-01-01

of the transfused blood. Therefore, we studied the relationship between blood storage time and the development of disease recurrence and long-term survival after colorectal cancer surgery. METHODS: Preoperative and postoperative data were prospectively recorded in 740 patients undergoing elective resection...... for primary colorectal cancer. None of the patients received preoperative or postoperative chemotherapy or radiation therapy. Endpoints were overall survival and disease recurrence in the subgroup of patients operated on with curative intention who also survived the first 30 days after operation. Storage......BACKGROUND: Perioperative blood transfusion and subsequent development of postoperative infectious complications may lead to poor prognosis of patients with colorectal cancer. It has been suggested that the development of postoperative infectious complications may be related to the storage time...

Effect of dietary fat type on the fatty acids composition of irradiated and frozen storage japanese quails meat

International Nuclear Information System (INIS)

Abd EI-Wahab, S. A.

2009-01-01

The effect of substitution of dietary cotton seed oil (CSO) by used restaurant oil (URO) with different percentages 25% group 2 (G2), 50% group 3 (G3) and 100% group 4 (G4) in Japanese quail diets on the fatty acids composition of their meat especially polyunsaturated fatty acids (PUFA). The effect of gamma irradiation doses (1.5, 3 and 5 kGy) at frozen storage -18 C (degree) for 2 and 4 months in comparison with unirradiated and un storage were studied. The total saturated fatty acids (SFA) in quail meat fed G4 diet (100% URO) increased significantly in comparison with SFA in G2 (25% URO) and G3 (50% URO) but there is no significant effect with G2 and G3 in comparison with G1 (100% CSO).The monounsaturated fatty acids (MUFA) and polyunsaturated fatty acids (PUFA) were not affected by G2 and G3 diet. Also, linoleic acid (C 18:2, n-6) had the same trend in those groups with range (32.75% to 33.35%). It is concluded that feeding a diet with URO 25% and 50% conserve the content of linoleic acid and the content of PUFA in quail meat. The irradiation doses and storage periods had no significant effect on the linoleic acid, MUFA and PUFA content.

Kinetics of ascorbic acid degradation in un-pasteurized Iranian lemon juice during regular storage conditions.

Science.gov (United States)

Abbasi, A; Niakousari, M

2008-05-15

The aim of this research was to determine shelf life stability of un-pasteurized lemon juice filled in clear or dark green glass bottles. Presence of light, time and temperature affect the ascorbic acid retention in citrus juices. Bottles were stored at room temperature (27 +/- 3 degrees C) and in the refrigerator (3 +/- 1 degrees C). Total soluble solids, total titrable acidity and pH value were measured every three weeks and analysis was carried out on ascorbic acid content by means of titration method in the presence of 2,6-dichlorophenol indophenol. The study was carried out for 12 weeks after which slight changes in color, taste and apparent texture in some samples were observed and ascorbic acid content reduced by 50%. Soluble solids content, pH value and total acidity were 5.5 degrees Brix, 2.73 and 5 g/100 mL, respectively which appeared not to be significantly influenced by storage time or conditions. Ascorbic acid content initially at 38.50 mg/100 mL was sharply reduced to about 22 mg/100 mL within the first three weeks of storage. The final ascorbic acid content of all samples was about 15 mg/100 mL. The deteriorative reaction of ascorbic acid in the juice at all conditions followed a first-order kinetic model with activation energy of 137 cal mol(-1).

Influence of Frozen Storage on the Fatty Acid Composition of Ostrich ...

African Journals Online (AJOL)

The aim of the study was to evaluate the effect of the duration (24 hours, 60 days and 120 days) of frozen storage (−20 ºC) on the fatty acid composition of meat from ostriches supplemented with linseed and rapeseed. The study was carried out on muscles of 40 ostriches raised on five dietary groups: control with no ...

Quantification of free and total sialic acid excretion by LC-MS/MS

NARCIS (Netherlands)

van der Ham, Maria; Prinsen, Berthil H. C. M. T.; Huijmans, Jan G. M.; Abeling, Nicolaas G. G. M.; Dorland, Bert; Berger, Ruud; de Koning, Tom J.; de Sain-van der Velden, Monique G. M.

2007-01-01

The main purpose for measuring urinary free sialic acid (FSA) is to diagnose sialic acid (SA) storage diseases. Elevated amounts of conjugated sialic acid (CSA) are observed in several diseases indicating the need to quantify CSA as well. A LC-MS/MS method for quantification of FSA and total sialic

Metabolic encephalopathy and lipid storage myopathy associated with a presumptive mitochondrial fatty acid oxidation defect in a dog

Directory of Open Access Journals (Sweden)

Vanessa R Biegen

2015-11-01

Full Text Available A 1-year-old spayed female Shih Tzu presented for episodic abnormalities of posture and mentation. Neurologic examination was consistent with a bilaterally symmetric multifocal encephalopathy. The dog had a waxing-and-waning hyperlactemia and hypoglycemia. Magnetic resonance imaging revealed bilaterally symmetric cavitated lesions of the caudate nuclei with less severe abnormalities in the cerebellar nuclei. Empirical therapy was unsuccessful and the patient was euthanized. Post-mortem histopathology revealed bilaterally symmetric necrotic lesions of the caudate and cerebellar nuclei and multi-organ lipid accumulation, including a lipid storage myopathy. Malonic aciduria and ketonuria were found on urinary organic acid screen. Plasma acylcarnitine analysis suggested a fatty acid oxidation defect. Fatty acid oxidation disorders are inborn errors of metabolism documented in humans, but poorly described in dogs. Although neurologic signs have been described in humans with this group of diseases, descriptions of advanced imaging and histopathology are severely lacking. This report suggests that abnormalities of fatty acid metabolism may cause severe, bilateral gray matter necrosis and lipid accumulation in multiple organs including the skeletal muscles, liver, and kidneys. Veterinarians should be aware that fatty acid oxidation disorders, although potentially fatal, may be treatable. A timely definitive diagnosis is essential in guiding therapy.

A novel mutation in PNPLA2 leading to neutral lipid storage disease with myopathy.

Science.gov (United States)

Ash, Daniel B; Papadimitriou, Dimitra; Hays, Arthur P; Dimauro, Salvatore; Hirano, Michio

2012-09-01

Mutations in PNPLA2, a gene encoding adipose triglyceride lipase, lead to neutral lipid storage disease with myopathy. To report the clinical and molecular features of a case of neutral lipid storage disease with myopathy resulting from a novel mutation in PNPLA2. Case report. University hospital. A 65-year-old man with progressive muscle weakness and high serum creatine kinase levels. Direct sequencing of the PNPLA2 gene. Identification of a novel homozygous mutation in the patient's PNPLA2 gene confirmed the suspected diagnosis of neutral lipid storage disease with myopathy. Screening of the PNPLA2 gene should be considered for patients presenting with high levels of creatine kinase, progressive muscle weakness, and systemic lipid accumulation. The presence of Jordans anomaly can be a strong diagnostic clue.

Effects of alpha-lipoic acids on sperm membrane integrity during liquid storage of boar semen

Directory of Open Access Journals (Sweden)

Laura Parlapan

2015-05-01

Full Text Available Preliminary studies have shown that sperm membrane from swine shows high sensitivity to cryopreservation process, causing a dramatic reduction in sperm quality. This has been attributed to the production of reactive oxygen species, that cause lipid peroxidation in sperm membranes. The aim of the present study was to minimize the oxidative attack by adding different concentration of alpha-lipoic acid into the sperm liquid storage at 17ºC for 7 days. Freshly ejaculated boar semen was diluted with Beltsville Thawing Solution (BTS and supplemented with 5 levels of alpha-lipoic  acid (0.015, 0.02, 0.05, 0.1, 0.15 mmol/ml. The membrane integrity was evaluated at days 0, 1, 3, 5 and 7 of liquid preservation, using flow cytometer FACSCanto II (BD Biociencias systems. The experiment indicate that supplementation of alpha-lipoic  acid to the semen liquid storage extender improve sperm membrane

Lithium-storage Properties of Gallic Acid-Reduced Graphene Oxide and Silicon-Graphene Composites

International Nuclear Information System (INIS)

Xu, Binghui; Zhang, Jintao; Gu, Yi; Zhang, Zhi; Al Abdulla, Wael; Kumar, Nanjundan Ashok; Zhao, X.S.

2016-01-01

Graphene oxide (GO) was de-oxygenated using gallic acid under mild conditions to prepare reduced graphene oxide (RGO). The resultant RGO showed a lithium-ion storage capacity of 1280 mA h g −1 at a current density of 200 mA g −1 after 350 cycles when used as an anode for lithium ion batteries. The RGO was further used to stabilize silicon (Si) nanoparticles to prepare silicon-graphene composite electrode materials. Experimental results showed that a composite electrode prepared with a mass ratio of Si:GO = 1:2 exhibited the best lithium ion storage performance.

Effect of storage and cooking on the fatty acid profile of omega-3 enriched eggs and pork meat marketed in Belgium.

Science.gov (United States)

Douny, Caroline; El Khoury, Rawad; Delmelle, Julien; Brose, François; Degand, Guy; Moula, Nassim; Farnir, Frédéric; Clinquart, Antoine; Maghuin-Rogister, Guy; Scippo, Marie-Louise

2015-03-01

The fatty acids (FA) profile was determined in n-3 enriched (Columbus™) Belgian eggs and pork in order to evaluate to what extent the n-3 fatty acids, which are very sensitive to oxidation, are resistant to storage or cooking. In standard eggs or pork, no change of the fatty acid profile was observed after storage or cooking without culinary fat, as well as in Columbus™ eggs and pork after storage. Some cooking processes (eggs in custard and meat in oven) induced a slight significant loss of n-3 fatty acids in Columbus™ eggs or pork (11.1% in fat from eggs cooked in custard vs. 15.3% in raw Columbus™ eggs and 11.0% in fat from oven cooked meat vs. 11.6% in raw Columbus™ meat). As expected, when Columbus™ pork is cooked with culinary fat, its fatty acid profile is modified according to the nature of the fat used.

Evidence for conjugated linoleic acid-induced embryonic mortality that is independent of egg storage conditions and changes in egg relative fatty acids.

Science.gov (United States)

Leone, V A; Stransky, D L; Aydin, R; Cook, M E

2009-09-01

Three experiments were performed to determine the effect of conjugated linoleic acid (CLA) on embryonic development in the absence of vitelline membrane disruption. In experiment 1, when eggs from control and CLA (0.5%)-fed hens were stored at 21 or 15 degrees C for 48 h, mineral movement between the yolk and albumen was not observed (with the exception of Mg and Na). Also, it was found that CLA-induced changes in yolk fatty acid content (e.g., increased saturated fatty acids and CLA) had begun to change after 5 d of feeding hens CLA, and no differences were detected in fatty acid composition after 14 d. In experiment 2, the hatchability of eggs incubated directly after oviposition or stored 24 h at 21 or 15 degrees C was determined from hens fed control or 0.5% CLA diets. Regardless of storage conditions, CLA reduced hatchability. These data showed that CLA elicits negative effects on hatchability independent of vitelline membrane disruption or egg storage condition. In experiment 3, eggs were collected from hens fed 0 or 1% CLA daily for 3 wk, stored at 21 degrees C for 24 h, and incubated. Not only did CLA decrease hatchability, the data showed as the days of CLA feeding increased, the days of survival during incubation decreased. Average days of embryonic survival during incubation for the CLA group diminished to 18.0, 13.4, and 6.3 d for wk 1, 2, and 3 of CLA feeding, respectively, and control remained at 20.6, 20.8, and 19.8 for the 3 wk. These studies suggested that without the disruption of the vitelline membrane, hatchability and embryonic days of survival were significantly reduced by maternal CLA feeding in comparison to control-fed hens. Evidence that embryos die earlier the longer the hens are fed CLA, even though no additional changes in the fatty acid content of eggs were found, suggested that factors other than storage and egg yolk fatty acid composition played a role in CLA-induced embryonic mortality.

Homogeneous Catalysis for Sustainable Hydrogen Storage in Formic Acid and Alcohols.

Science.gov (United States)

Sordakis, Katerina; Tang, Conghui; Vogt, Lydia K; Junge, Henrik; Dyson, Paul J; Beller, Matthias; Laurenczy, Gábor

2018-01-24

Hydrogen gas is a storable form of chemical energy that could complement intermittent renewable energy conversion. One of the main disadvantages of hydrogen gas arises from its low density, and therefore, efficient handling and storage methods are key factors that need to be addressed to realize a hydrogen-based economy. Storage systems based on liquids, in particular, formic acid and alcohols, are highly attractive hydrogen carriers as they can be made from CO 2 or other renewable materials, they can be used in stationary power storage units such as hydrogen filling stations, and they can be used directly as transportation fuels. However, to bring about a paradigm change in our energy infrastructure, efficient catalytic processes that release the hydrogen from these molecules, as well as catalysts that regenerate these molecules from CO 2 and hydrogen, are required. In this review, we describe the considerable progress that has been made in homogeneous catalysis for these critical reactions, namely, the hydrogenation of CO 2 to formic acid and methanol and the reverse dehydrogenation reactions. The dehydrogenation of higher alcohols available from renewable feedstocks is also described. Key structural features of the catalysts are analyzed, as is the role of additives, which are required in many systems. Particular attention is paid to advances in sustainable catalytic processes, especially to additive-free processes and catalysts based on Earth-abundant metal ions. Mechanistic information is also presented, and it is hoped that this review not only provides an account of the state of the art in the field but also offers insights into how superior catalytic systems can be obtained in the future.

Microbial Profile of Supragingival and Subgingival Plaque of Patients With Glycogen Storage Disease

Directory of Open Access Journals (Sweden)

Chealsea E. Garcia DDS, MS

2016-12-01

Full Text Available Patients with glycogen storage disease (GSD are either orally fed (ORF or gastronomy-tube fed (GTF with cornstarch to maintain normal glucose levels. It is not known whether the use of cornstarch affects the microbiological oral profile of patients with GSD. Thus, the purpose of this study was to compare supragingival and subgingival plaque samples collected from 53 participants with GSD (2-56 years—29 ORF and 24 GTF. The 16S sequence bacterial profiles of plaque DNA were obtained and a total of 768 probes were detected across the plaque groups. Orally fed patients showed higher means of cariogenic species and periodontal health-associated species, whereas GTF patients showed higher means of periopathogenic species ( P < .05. Orally fed patients exhibited high levels of caries pathogens and lower levels of periodontal pathogens possibly due to the acidic environment created by their cornstarch diet, when compared to GTF patients.

Study of structural, surface and hydrogen storage properties of boric acid mediated metal (sodium)-organic frameworks

Science.gov (United States)

Ozer, Demet; Köse, Dursun A.; Sahin, Onur; Oztas, Nursen A.

2018-04-01

Three boric acid mediated metal organic frameworks were synthesized by solution method with using succinic acid, fumaric acid and acetylene dicarboxylic acid as a ligand source and sodium as a metal source. The complexes were characterized by FT-IR, powder XRD, elemental analyses and single crystal measurements. The complexes with the formula, C4H18B2Na2O14, C4H16B2Na2O14 and C4H14B2Na2O14 were successfully obtained. BET surface area of complexes were calculated and found as 13.474 m2/g for catena-(tetrakis(μ2-hydroxo)-(μ2-trihydrogen borate)-(μ2-succinato)-di-sodium boric acid solvate), 1.692 m2/g for catena-(tetrakis(μ2-hydroxo)-(μ2-trihydrogen borate)-(μ2-fumarato)-di-sodium boric acid solvate) and 5.600 m2/g for catena-(tetrakis(μ2-hydroxo)-(μ2-trihydrogen borate)-(μ2-acetylenedicarboxylato)-di-sodium boric acid solvate). Hydrogen storage capacities of the complexes were also studied at 77 K 1 bar pressure and found as 0.108%, 0.033%, 0.021% by mass. When different ligands were used, the pore volume, pore width and surface area of the obtained complexes were changed. As a consequence, hydrogen storage capacities also changed.

Dietary Management of the Ketogenic Glycogen Storage Diseases

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Kaustuv Bhattacharya MBBS, MRCPCH, FRACP, MD

2016-08-01

Full Text Available The glycogen storage diseases (GSDs comprise a group of rare inherited disorders of glycogen metabolism. The hepatic glycogenolytic forms of these disorders are typically associated with hypoglycemia and hepatomegaly. For GSD I, secondary metabolic disturbances include fasting hyperlactatemia, hyperuricemia, and hyperlipidemia. Glycogen storage disease III is caused by reduced activity of the debrancher enzyme, GSD VI by phosphorylase, and GSD IX by phosphorylase kinase. It has often been reported that the non-GSD I group of disorders have a benign course. However, myopathy, cardiomyopathy, and cirrhosis have been reported significant clinical morbidities associated with GSD III and IX in particular. There have been a range of reports indicating high-protein diets, high-fat diets, medium chain triglyceride (MCT, modified Atkins diet, and therapeutic ketones as rescuing severe phenotypes of GSD III in particular. The etiology of these severe phenotypes has not been defined. Cases presented in this report indicate potential harm from excessive simple sugar use in GSD IX C. Review of the literature indicates that most interventions have reduced the glycemic load and provide alternate substrates for energy in rescue situations. Prevention of complications is most likely to occur with a mixed balanced low glycemic index diet potentially with relative increases in protein.

CNS-directed gene therapy for lysosomal storage diseases

OpenAIRE

Sands, Mark S; Haskins, Mark E

2008-01-01

Lysosomal storage diseases (LSDs) are a group of inherited metabolic disorders usually caused by deficient activity of a single lysosomal enzyme. As most lysosomal enzymes are ubiquitously expressed, a deficiency in a single enzyme can affect multiple organ systems, including the central nervous system (CNS). At least 75% of all LSDs have a significant CNS component. Approaches such as bone marrow transplantation (BMT) or enzyme replacement therapy (ERT) can effectively treat the systemic dis...

Bile Acid Signaling in Metabolic Disease and Drug Therapy

Science.gov (United States)

Li, Tiangang

2014-01-01

Bile acids are the end products of cholesterol catabolism. Hepatic bile acid synthesis accounts for a major fraction of daily cholesterol turnover in humans. Biliary secretion of bile acids generates bile flow and facilitates hepatobiliary secretion of lipids, lipophilic metabolites, and xenobiotics. In the intestine, bile acids are essential for the absorption, transport, and metabolism of dietary fats and lipid-soluble vitamins. Extensive research in the last 2 decades has unveiled new functions of bile acids as signaling molecules and metabolic integrators. The bile acid–activated nuclear receptors farnesoid X receptor, pregnane X receptor, constitutive androstane receptor, vitamin D receptor, and G protein–coupled bile acid receptor play critical roles in the regulation of lipid, glucose, and energy metabolism, inflammation, and drug metabolism and detoxification. Bile acid synthesis exhibits a strong diurnal rhythm, which is entrained by fasting and refeeding as well as nutrient status and plays an important role for maintaining metabolic homeostasis. Recent research revealed an interaction of liver bile acids and gut microbiota in the regulation of liver metabolism. Circadian disturbance and altered gut microbiota contribute to the pathogenesis of liver diseases, inflammatory bowel diseases, nonalcoholic fatty liver disease, diabetes, and obesity. Bile acids and their derivatives are potential therapeutic agents for treating metabolic diseases of the liver. PMID:25073467

Testosterone-dependent sex differences in red blood cell hemolysis in storage, stress, and disease.

Science.gov (United States)

Kanias, Tamir; Sinchar, Derek; Osei-Hwedieh, David; Baust, Jeffrey J; Jordan, Andrew; Zimring, James C; Waterman, Hayley R; de Wolski, Karen S; Acker, Jason P; Gladwin, Mark T

2016-10-01

Red blood cell (RBC) hemolysis represents an intrinsic mechanism for human vascular disease. Intravascular hemolysis releases hemoglobin and other metabolites that inhibit nitric oxide signaling and drive oxidative and inflammatory stress. Although these pathways are important in disease pathogenesis, genetic and population modifiers of hemolysis, including sex, have not been established. We studied sex differences in storage or stress-induced hemolysis in RBC units from the United States and Canada in 22 inbred mouse strains and in patients with sickle cell disease (SCD) using measures of hemolysis in 315 patients who had homozygous SS hemoglobin from the Walk-PHASST cohort. A mouse model also was used to evaluate posttransfusion recovery of stored RBCs, and gonadectomy was used to determine the mechanisms related to sex hormones. An analysis of predisposition to hemolysis based on sex revealed that male RBCs consistently exhibit increased susceptibility to hemolysis compared with females in response to routine cold storage, under osmotic or oxidative stress, after transfusion in mice, and in patients with SCD. The sex difference is intrinsic to the RBC and is not mediated by plasmatic factors or female sex hormones. Importantly, orchiectomy in mice improves RBC storage stability and posttransfusion recovery, whereas testosterone repletion therapy exacerbates hemolytic response to osmotic or oxidative stress. Our findings suggest that testosterone increases susceptibility to hemolysis across human diseases, suggesting that male sex may modulate clinical outcomes in blood storage and SCD and establishing a role for donor genetic variables in the viability of stored RBCs and in human hemolytic diseases. © 2016 AABB.

Furan formation from fatty acids as a result of storage, gamma irradiation, UV-C and heat treatments

Science.gov (United States)

Furan is a possible human carcinogen that has been found in many thermally processed foods. The effects of thermal processing, gamma and UV-C irradiation on formation of furan from different fatty acids was studied. In addition, formation of furan from fatty acid emulsions during storage at 25C and...

Tree age, fruit size and storage conditions affect levels of ascorbic acid, total phenolic concentrations and total antioxidant activity of 'Kinnow' mandarin juice.

Science.gov (United States)

Khalid, Samina; Malik, Aman U; Khan, Ahmad S; Shahid, Muhammad; Shafique, Muhammad

2016-03-15

Bioactive compounds (ascorbic acid, total phenolics and total antioxidants) are important constituents of citrus fruit juice; however, information with regard to their concentrations and changes in relation to tree age and storage conditions is limited. 'Kinnow' (Citrus nobilis Lour × Citrus deliciosa Tenora) mandarin juice from fruit of three tree ages (6, 18 and 35 years old) and fruit sizes (large, medium and small) were examined for their bioactive compounds during 7 days under ambient storage conditions (20 ± 2 °C and 60-65% relative humidity (RH)) and during 60 days under cold storage (4 ± 1 °C and 75-80% RH) conditions. Under ambient conditions, a reduction in total phenolic concentrations (TPC) and in total antioxidant activity (TAA) was found for the juice from all tree ages and fruit sizes. Overall, fruit from 18-year-old trees had higher mean TPC (95.86 µg mL(-1) ) and TAA (93.68 mg L(-1) ), as compared to 6 and 35-year-old trees. Likewise, in cold storage, TAA decreased in all fruit size groups from 18 and 35-year-old trees. In all tree age and fruit size groups, TPC decreased initially during 15 days of cold storage and then increased gradually with increase in storage duration. Ascorbic acid concentrations showed an increasing trend in all fruit size groups from 35-year-old trees. Overall, during cold storage, fruit from 18-year-old trees maintained higher mean ascorbic acid (33.05 mg 100 mL(-1) ) concentrations, whereas fruit from 6-year-old trees had higher TAA (153.1 mg L(-1) ) and TPC (115.1 µg mL(-1) ). Large-sized fruit had higher ascorbic acid (32.08 mg 100 mL(-1) ) concentrations and TAA (157.5 mg L(-1) ). Fruit from 18-year-old trees maintained higher TPC and TAA under ambient storage conditions, whereas fruit from 6-year-old trees maintained higher TPC and TAA during cold storage. Small-sized fruit had higher TPC after ambient temperature storage, whereas large fruit size showed higher ascorbic acid concentrations and TAA after cold

Inhibition of substrate synthesis as a strategy for glycolipid lysosomal storage disease therapy

NARCIS (Netherlands)

Platt, F. M.; Jeyakumar, M.; Andersson, U.; Priestman, D. A.; Dwek, R. A.; Butters, T. D.; Cox, T. M.; Lachmann, R. H.; Hollak, C.; Aerts, J. M.; van Weely, S.; Hrebícek, M.; Moyses, C.; Gow, I.; Elstein, D.; Zimran, A.

2001-01-01

The glycosphingolipid (GSL) lysosomal storage diseases are caused by mutations in the genes encoding the glycohydrolases that catabolize GSLs within lysosomes. In these diseases the substrate for the defective enzyme accumulates in the lysosome and the stored GSL leads to cellular dysfunction and

Corn Crisps Enriched in Omega-3 Fatty Acids Sensory Characteristic and its Changes During Storage.

Science.gov (United States)

Rogalski, Mateusz; Nowak, Karolina; Fiedor, Piotr; Szterk, Arkadiusz

Extruded cereal snacks are usually deficient in protein, mineral ingredients, valuable fatty acids. With the rise of health awareness among consumers, food manufacturers and scientists are pressed to take measures in order to develop new functional/health-beneficial foods. The aim of this work was to manufacture extruded crisps enriched with α-linolenic acid (obtained from linseed oil) and to observe whether storage of the product for the period of 6 months would cause its disqualification, primarily due to its sensory properties and secondarily due to its chemical properties. The research demonstrated that the addition of linseed oil to corn crisps at the amount of 5 % enables to obtain functional corn crisps containing over 2 g of ALA in a portion of 100 g even after 6 months of storage at room temperature. ALA-enriched crisps maintain the original sensory profile after 6 months of storage and their sensory profile is similar to the profile of crisps without the addition of linseed oil if they are packed in barrier packaging filled 100 % with argon. Therefore, they may be a healthier alternative to typical corn crisps.

Effect of previous ascorbic acid treatment on the fatty acid profile of cobia (Rachycentron canadum fillets during frozen storage

Directory of Open Access Journals (Sweden)

Aftabsavar, Y.

2012-03-01

Full Text Available The present research focuses on the nutritional value of the lipid retention associated with frozen cobia (Rachycentron canadum. The effect of a previous soaking in an aqueous ascorbic acid (AA solution on the fatty acid profile of fish fillets during a further frozen storage period (–18 °C was investigated. Two different AA concentrations were tested (0.25% and 0.50% and compared to control conditions. As a result of the frozen storage period (up to 6 months, marked decreases were found in the contents of fatty acid groups such as monounsaturated, polyunsaturated and n-3 polyunsaturated, as well as in the n-3/n-6 ratio. However, a preserving effect on such fatty acid parameters could be observed resulting from the previous AA treatment, which was greater when applying the 0.50% AA concentration. Assessment of the polyene index indicated an increased lipid oxidation development during the frozen storage time; this increase was partially inhibited by the previous AA soaking.Este estudio se centra en el valor nutricional lipídico de cobia (Rachycentron canadum congelada. Para ello, se investiga el efecto que un tratamiento previo con ácido ascórbico (AA puede tener sobre el perfil de ácidos grasos de filete de pescado durante su conservación en congelación (–18 °C; seis meses. Se aplicaron dos concentraciones de AA (0.25% y 0.50% que fueron comparadas con muestras control. Como resultado de la conservación en congelación, se observó un descenso importante en grupos de ácidos grasos monoinsaturados, poliinsaturados y poliinsaturados de las serie n-3, así como en la relación n-3/n-6. Sin embargo, el tratamiento previo con AA produjo un efecto protector en estos parámetros, siendo mayor al aplicar AA en la concentración superior. La medida del índice de polienos reflejó un incremento de la oxidación lipídica durante la conservación en congelación; este incremento fue parcialmente inhibido como resultado del tratamiento

Effect of pressing and combination of three storage temperatures and times on chemical composition and fatty acid profile of canola expellers

Directory of Open Access Journals (Sweden)

Matteo Guadagnin

2013-05-01

Full Text Available This experiment investigated the effects of combinations of three temperatures and storage times on chemical composition, fatty acid profile, and oxidative stability of canola expellers obtained from the cold-pressing extraction of oil. Canola seeds were single-crushed at moderate temperatures (60°C during 3 pressing sessions. Nine samples (100±1 g of each session were collected, inserted into sealed bags, stored at three temperatures (12, 24, 36°C over 3 periods of time (10, 20, 30 d. Then, samples (100±1 g of canola seeds collected before each pressing session and canola expellers collected before and after each storage time were analyzed for chemical composition, fatty acid profile, peroxide number and Kreis test. Before storage, the fatty acid profile of canola seeds and expellers differed significantly, except for myristic (P=0.18, palmitic (P=0.57, oleic (P=0.07, and α-linolenic acids (P=0.45. Compared to canola seeds, expellers showed greater content of saturated, poly-unsaturated, and n-6 fatty acids (P<0.01, but a lower content of mono-unsaturated fatty acids (P<0.01. Peroxide values were definitely (P<0.01 greater for expellers and averaged 4.22 and 4.11 mEq/kg fat before and after storage, respectively. The Kreis test was negative for all samples. Under different temperatures and times of storage, canola expellers showed to maintain a good oxidative stability, as highlighted by low peroxide values (<10 mEq/kg fat and negative response for Kreis test. Canola expellers obtained by on-farm cold extraction, despite great oil residual (from 17 to 19% ether extracts on dry matter basis, can be stored at farm without significant chemical and nutritional changes.

Pulmonary Arterial Hypertension in Glycogen Storage Disease Type I

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Rachel D. Torok MD

2017-05-01

Full Text Available Pulmonary arterial hypertension (PAH is a rare and highly fatal disease that has been reported in 8 patients with glycogen storage disease type I (GSDI. We describe an additional case of an acute presentation of PAH in a 14-year-old patient with GSDI, which was successfully treated with inhaled nitric oxide and sildenafil. We investigated the incidence of PAH in 28 patients with GSDI on routine echocardiography and found no evidence of PAH and no significant cardiac abnormalities. This study highlights that PAH is a rare disease overall, but our case report and those previously described suggest an increased incidence in patients with GSDI. Should cardiopulmonary symptoms develop, clinicians caring for patients with GSDI should have a high degree of suspicion for acute PAH and recognize that prompt intervention can lead to survival in this otherwise highly fatal disease.

A multifunctional energy-storage system with high-power lead-acid batteries

Science.gov (United States)

Wagner, R.; Schroeder, M.; Stephanblome, T.; Handschin, E.

A multifunctional energy storage system is presented which is used to improve the utilization of renewable energy supplies. This system includes three different functions: (i) uninterruptible power supply (UPS); (ii) improvement of power quality; (iii) peak-load shaving. The UPS application has a long tradition and is used whenever a reliable power supply is needed. Additionally, nowadays, there is a growing demand for high quality power arising from an increase of system perturbation of electric grids. Peak-load shaving means in this case the use of renewable energy stored in a battery for high peak-load periods. For such a multifunctional application large lead-acid batteries with high power and good charge acceptance, as well as good cycle life are needed. OCSM batteries as with positive tubular plates and negative copper grids have been used successfully for a multitude of utility applications. This paper gives two examples where multifunctional energy storage systems have started operation recently in Germany. One system was installed in combination with a 1 MW solar plant in Herne and another one was installed in combination with a 2 MW wind farm in Bocholt. At each place, a 1.2 MW h (1 h-rate) lead-acid battery has been installed. The batteries consist of OCSM cells with the standard design but modified according to the special demand of a multifunctional application.

Preparation, characterization, and thermal properties of starch microencapsulated fatty acids as phase change materials thermal energy storage applications

Science.gov (United States)

Stable starch-oil composites can be prepared from renewable resources by excess steam jet-cooking aqueous slurries of starch and vegetable oils or other hydrophobic materials. Fatty acids such as stearic acid are promising phase change materials (PCMs) for latent heat thermal energy storage applica...

Effects of a propionic-acid based preservative on storage characteristics of alfalfa-orchardgrass hay in large-rectangular bales

Science.gov (United States)

For many years, various formulations of organic acids have been marketed as preservatives, most specifically for use on hays that could not be field-dried to moisture concentrations low enough to reduce or eliminate spontaneous heating during storage. These preservatives are often propionic-acid-bas...

Acid perfusion test in gastroesophageal reflux disease

Energy Technology Data Exchange (ETDEWEB)

Kaul, B.; Petersen, H.; Grette, K.; Myrvold, H.E.

1986-01-01

An acid perfusion test, isotope scanning, endoscopy, and esophageal biopsy were performed in 101 patients with symptoms strongly suggestive of gastroesophageal reflux (GER) disease. A positive acid perfusion test within 30 min (APT) and within 5 min (TAPT) was found in 70.2% and 37.6% of the patients, respectively. A positive APT was found significantly more often in patients with than without endoscopic esophagitis, whereas a positive TAPT was found significantly more often in patients with severe symptoms than in patients with moderate symptoms, and in a significantly higher proportion of patients with than without GER by scintigraphy. Neither the APT nor the TAPT showed any dependency on the presence of histologic esophagitis. Most (97%) patients with a negative acid perfusion test, in addition to typical symptoms, also presented with scintigraphic, endoscopic, or histologic evidence of GER disease. Although it shows that the acid perfusion test, particularly when early positive, may serve as a weak predictor of the severity of GER disease, the present study gives little support to the test's clinical usefulness.

Lipid Involvement in Neurodegenerative Diseases of the Motor System: Insights from Lysosomal Storage Diseases.

Science.gov (United States)

Dodge, James C

2017-01-01

Lysosomal storage diseases (LSDs) are a heterogeneous group of rare inherited metabolic diseases that are frequently triggered by the accumulation of lipids inside organelles of the endosomal-autophagic-lysosomal system (EALS). There is now a growing realization that disrupted lysosomal homeostasis (i.e., lysosomal cacostasis) also contributes to more common neurodegenerative disorders such as Parkinson disease (PD). Lipid deposition within the EALS may also participate in the pathogenesis of some additional neurodegenerative diseases of the motor system. Here, I will highlight the lipid abnormalities and clinical manifestations that are common to LSDs and several diseases of the motor system, including amyotrophic lateral sclerosis (ALS), atypical forms of spinal muscular atrophy, Charcot-Marie-Tooth disease (CMT), hereditary spastic paraplegia (HSP), multiple system atrophy (MSA), PD and spinocerebellar ataxia (SCA). Elucidating the underlying basis of intracellular lipid mislocalization as well as its consequences in each of these disorders will likely provide innovative targets for therapeutic research.

Differential response of the liver to bile acid treatment in a mouse model of Niemann-Pick disease type C [version 2; referees: 2 approved, 1 not approved

Directory of Open Access Journals (Sweden)

Elena-Raluca Nicoli

2018-04-01

Full Text Available Niemann-Pick disease type C (NPC disease is a neurodegenerative lysosomal storage disease caused by mutations in the NPC1 or NPC2 genes. Liver disease is also a common feature of NPC that can present as cholestatic jaundice in the neonatal period. Liver enzymes can remain elevated above the normal range in some patients as they age. We recently reported suppression of the P450 detoxification system in a mouse model of NPC disease and also in post-mortem liver from NPC patients. We demonstrated the ability of the hydrophobic bile acid ursodeoxycholic acid (UDCA (3α, 7β-dihydroxy-5β-cholanic acid to correct the P450 system suppression. UDCA is used to treat several cholestatic disorders and was tested in NPC due to the P450 system being regulated by bile acids. Here, we compare the effect of UDCA and cholic acid (CA, another bile acid, in the NPC mouse model. We observed unexpected hepatotoxicity in response to CA treatment of NPC mice. No such hepatotoxicity was associated with UDCA treatment. These results suggest that CA treatment is contraindicated in NPC patients, whilst supporting the use of UDCA as an adjunctive therapy in NPC patients.

Storage time of transfused blood and disease recurrence after colorectal cancer surgery

DEFF Research Database (Denmark)

Mynster, T; Nielsen, Hans Jørgen

2001-01-01

of the transfused blood. Therefore, we studied the relationship between blood storage time and the development of disease recurrence and long-term survival after colorectal cancer surgery. METHODS: Preoperative and postoperative data were prospectively recorded in 740 patients undergoing elective resection......BACKGROUND: Perioperative blood transfusion and subsequent development of postoperative infectious complications may lead to poor prognosis of patients with colorectal cancer. It has been suggested that the development of postoperative infectious complications may be related to the storage time...... transfused patients (P = 0.004). The survival of patients receiving blood exclusively stored blood stored > or = 21 days, survival was 3.7 years (P = 0.12). Among patients with curative resection (n = 532), the hazard ratio of disease recurrence was 1.5 (95...

Thermal properties and thermal reliability of eutectic mixtures of some fatty acids as latent heat storage materials

International Nuclear Information System (INIS)

Sari, Ahmet; Sari, Hayati; Oenal, Adem

2004-01-01

The present study deals with two subjects. The first one is to determine the thermal properties of lauric acid (LA)-stearic acid (SA), myristic acid (MA)-palmitic acid (PA) and palmitic acid (PA)-stearic acid (SA) eutectic mixtures as latent heat storage material. The properties were measured by the differential scanning calorimetry (DSC) analysis technique. The second one is to study the thermal reliability of these materials in view of the change in their melting temperatures and latent heats of fusion with respect to repeated thermal cycles. For this aim, the eutectic mixtures were subjected to 360 repeated melt/freeze cycles, and their thermal properties were measured after 0, 90,180 and 360 thermal cycles by the technique of DSC analysis. The DSC thermal analysis results show that the binary systems of LA-SA in the ratio of 75.5:24.5 wt.%, MA-PA in the ratio of 58:42 wt.% and PA-SA in the ratio of 64.2:35.8 wt.% form eutectic mixtures with melting temperatures of 37.0, 42.60 and 52.30 deg. C and with latent heats of fusion of 182.7, 169.7 and 181.7 J g -1 , respectively. These thermal properties make them possible for heat storage in passive solar heating applications with respect to climate conditions. The accelerated thermal cycle tests indicate that the changes in the melting temperatures and latent heats of fusion of the studied eutectic mixtures are not regular with increasing number of thermal cycles. However, these materials, latent heat energy storage materials, have good thermal reliability in terms of the change in their thermal properties with respect to thermal cycling for about a one year utility period

Lumbar gibbus in storage diseases and bone dysplasias

Energy Technology Data Exchange (ETDEWEB)

Levin, T.L. [Department of Radiology, Division of Pediatric Radiology, Columbia-Presbyterian Medical Center, Babies and Children`s Hospital of New York, NY (United States); Berdon, W.E. [Department of Radiology, Division of Pediatric Radiology, Columbia-Presbyterian Medical Center, Babies and Children`s Hospital of New York, NY (United States); Lachman, R.S. [International Skeletal Dysplasia Registry, Los Angeles, CA (United States); Anyane-Yeboa, K. [Department of Pediatrics, Columbia-Presbyterian Medical Center, Babies and Children`s Hospital of New York, NY (United States); Ruzal-Shapiro, C. [Department of Radiology, Division of Pediatric Radiology, Columbia-Presbyterian Medical Center, Babies and Children`s Hospital of New York, NY (United States); Roye, D.P. Jr. [Department of Orthopedic Surgery, Columbia-Presbyterian Medical Center, Babies and Children`s Hospital of New York, NY (United States)

1997-04-01

Objective. The objective of this study was to review the problem of lumbar gibbus in children with storage diseases and bone dysplasias utilizing plain films and MR imaging. Materials and methods. Clinical histories and radiographic images in five patients with storage diseases [four mucopolysaccharidosis (MPS) and one mucolipidosis] and two with achondroplasia were reviewed. The International Skeletal Dysplasia Registry (Los Angeles, Calif.), surveyed for all patients with lumbar gibbus and skeletal dysplasias, provided 12 additional cases. Results. All patients had localized gibbus of the upper lumbar spine, characterized by anterior wedging and posterior displacement of the vertebrae at the apex of the curve, producing a beaked appearance. The curve, exaggerated in the sitting or standing position, was most severe in the two patients with MPS-IV (one of whom died). Both developed severe neurologic signs and symptoms requiring surgical intervention. In four patients, MR images demonstrated the apex of the curve to be at or below the conus. Two patients demonstrated anterior herniation of the intervertebral discs at the apex of the curve, though the signal intensity of the intervertebral discs was normal. Conclusion. Lumbar gibbus has important neurologic and orthopedic implications, and is most severe in patients with MPS. The etiology of the gibbus with vertebral beaking is multifactorial and includes poor truncal muscle tone, weight-bearing forces, growth disturbance and anterior disc herniation. The curve is generally at or below the conus. Neurologic complications are unusual, although orthopedic problems can arise. Due to their longer survival, patients with achondroplasia or Morquio`s disease are more vulnerable to eventual gibbus-related musculoskeletal complications. (orig.). With 6 figs., 2 tabs.

Lumbar gibbus in storage diseases and bone dysplasias

International Nuclear Information System (INIS)

Levin, T.L.; Berdon, W.E.; Lachman, R.S.; Anyane-Yeboa, K.; Ruzal-Shapiro, C.; Roye, D.P. Jr.

1997-01-01

Objective. The objective of this study was to review the problem of lumbar gibbus in children with storage diseases and bone dysplasias utilizing plain films and MR imaging. Materials and methods. Clinical histories and radiographic images in five patients with storage diseases [four mucopolysaccharidosis (MPS) and one mucolipidosis[ and two with achondroplasia were reviewed. The International Skeletal Dysplasia Registry (Los Angeles, Calif.), surveyed for all patients with lumbar gibbus and skeletal dysplasias, provided 12 additional cases. Results. All patients had localized gibbus of the upper lumbar spine, characterized by anterior wedging and posterior displacement of the vertebrae at the apex of the curve, producing a beaked appearance. The curve, exaggerated in the sitting or standing position, was most severe in the two patients with MPS-IV (one of whom died). Both developed severe neurologic signs and symptoms requiring surgical intervention. In four patients, MR images demonstrated the apex of the curve to be at or below the conus. Two patients demonstrated anterior herniation of the intervertebral discs at the apex of the curve, though the signal intensity of the intervertebral discs was normal. Conclusion. Lumbar gibbus has important neurologic and orthopedic implications, and is most severe in patients with MPS. The etiology of the gibbus with vertebral beaking is multifactorial and includes poor truncal muscle tone, weight-bearing forces, growth disturbance and anterior disc herniation. The curve is generally at or below the conus. Neurologic complications are unusual, although orthopedic problems can arise. Due to their longer survival, patients with achondroplasia or Morquio's disease are more vulnerable to eventual gibbus-related musculoskeletal complications. (orig.). With 6 figs., 2 tabs

Disease Modeling and Gene Therapy of Copper Storage Disease in Canine Hepatic Organoids

Directory of Open Access Journals (Sweden)

Sathidpak Nantasanti

2015-11-01

Full Text Available The recent development of 3D-liver stem cell cultures (hepatic organoids opens up new avenues for gene and/or stem cell therapy to treat liver disease. To test safety and efficacy, a relevant large animal model is essential but not yet established. Because of its shared pathologies and disease pathways, the dog is considered the best model for human liver disease. Here we report the establishment of a long-term canine hepatic organoid culture allowing undifferentiated expansion of progenitor cells that can be differentiated toward functional hepatocytes. We show that cultures can be initiated from fresh and frozen liver tissues using Tru-Cut or fine-needle biopsies. The use of Wnt agonists proved important for canine organoid proliferation and inhibition of differentiation. Finally, we demonstrate that successful gene supplementation in hepatic organoids of COMMD1-deficient dogs restores function and can be an effective means to cure copper storage disease.

Rainbow trout (Salmo irideus produced in Finland I. Bacterial spoilage and amino acid composition of fresh rainbow trout during refrigerated storage

Directory of Open Access Journals (Sweden)

Fritz P. Niinivaara

1966-12-01

Full Text Available Bacteriological spoilage, organoleptical quality and amino acid composition of fresh trout were studied during storage at +4– +6° C. Experiments were carried out with living fish (control, with fish 4 hours after killing and during storage. The fish were kept in air, in ice and packed in polyethylene and vacuum bags. It was observed that the type of packing considerably influences both the bacteriological and organoleptical quality. These changes were not, however, directly correlated with each other. In connection with vacuum packing, the amounts of anaerobic sulphide producing bacteria were so high that this aspect needs a detailed investigation before vacuum packing can be recommended for fresh trout. The amino acid composition of iced trout changed only slightly during storage. Current experiments concerning changes in volatile amino acid contents will provide additional information in this respect.

Glycogen storage disease type III: modified Atkins diet improves myopathy.

Science.gov (United States)

Mayorandan, Sebene; Meyer, Uta; Hartmann, Hans; Das, Anibh Martin

2014-11-28

Frequent feeds with carbohydrate-rich meals or continuous enteral feeding has been the therapy of choice in glycogen storage disease (Glycogenosis) type III. Recent guidelines on diagnosis and management recommend frequent feedings with high complex carbohydrates or cornstarch avoiding fasting in children, while in adults a low-carb-high-protein-diet is recommended. While this regimen can prevent hypoglycaemia in children it does not improve skeletal and heart muscle function, which are compromised in patients with glycogenosis IIIa. Administration of carbohydrates may elicit reactive hyperinsulinism, resulting in suppression of lipolysis, ketogenesis, gluconeogenesis, and activation of glycogen synthesis. Thus, heart and skeletal muscle are depleted of energy substrates. Modified Atkins diet leads to increased blood levels of ketone bodies and fatty acids. We hypothesize that this health care intervention improves the energetic balance of muscles. We treated 2 boys with glycogenosis IIIa aged 9 and 11 years with a modified Atkins diet (10 g carbohydrate per day, protein and fatty acids ad libitum) over a period of 32 and 26 months, respectively. In both patients, creatine kinase levels in blood dropped in response to Atkins diet. When diet was withdrawn in one of the patients he complained of chest pain, reduced physical strength and creatine kinase levels rapidly increased. This was reversed when Atkins diet was reintroduced. One patient suffered from severe cardiomyopathy which significantly improved under diet. Patients with glycogenosis IIIa benefit from an improved energetic state of heart and skeletal muscle by introduction of Atkins diet both on a biochemical and clinical level. Apart from transient hypoglycaemia no serious adverse effects were observed.

Dietary fatty acids and cardiovascular disease: A review

Directory of Open Access Journals (Sweden)

Raquel Eccel Prates

2015-09-01

Full Text Available Fatty acids (FAs can be classified into saturated (SFA, unsaturated (poly- or monounsaturated and trans FA. Recent studies have found that both the quantity and quality of dietary FAs may influence their role in metabolic pathways. Due to their chemical composition, some FAs play a major role in the development and progression of cardiovascular disease. This is especially true for SFA and n-3 polyunsaturated fatty acids, which include marine eicosapentaenoic acid (EPA and docosahexaenoic acid (DHA. The proinflammatory effects of high SFA intake may increase the risk of atherosclerosis. On the other hand, dietary n-3 intake may reduce the risk of cardiovascular disease by decreasing atherosclerosis, inflammation, and thrombotic processes. The goal of this study was to review the current literature on the role of FA intake in the prevention and risk of cardiovascular disease.

Glycogen branching enzyme (GBE1) mutation causing equine glycogen storage disease IV.

Science.gov (United States)

Ward, Tara L; Valberg, Stephanie J; Adelson, David L; Abbey, Colette A; Binns, Matthew M; Mickelson, James R

2004-07-01

Comparative biochemical and histopathological evidence suggests that a deficiency in the glycogen branching enzyme, encoded by the GBE1 gene, is responsible for a recently identified recessive fatal fetal and neonatal glycogen storage disease (GSD) in American Quarter Horses termed GSD IV. We have now derived the complete GBE1 cDNA sequences for control horses and affected foals, and identified a C to A substitution at base 102 that results in a tyrosine (Y) to stop (X) mutation in codon 34 of exon 1. All 11 affected foals were homozygous for the X34 allele, their 11 available dams and sires were heterozygous, and all 16 control horses were homozygous for the Y34 allele. The previous findings of poorly branched glycogen, abnormal polysaccharide accumulation, lack of measurable GBE1 enzyme activity and immunodetectable GBE1 protein, coupled with the present observation of abundant GBE1 mRNA in affected foals, are all consistent with the nonsense mutation in the 699 amino acid GBE1 protein. The affected foal pedigrees have a common ancestor and contain prolific stallions that are likely carriers of the recessive X34 allele. Defining the molecular basis of equine GSD IV will allow for accurate DNA testing and the ability to prevent occurrence of this devastating disease affecting American Quarter Horses and related breeds.

Oxidative stability during storage of structured lipids produced from fish oil and caprylic acid

DEFF Research Database (Denmark)

Nielsen, Nina Skall; Xu, Xuebing; Timm Heinrich, Maike

2004-01-01

Structured lipids produced by enzymatic or chemical methods for different applications have been receiving considerable attention. The oxidative stability of a randomized structured lipid (RFO), produced by chemical interesterification from fish oil (FO) and tricaprylin, and a specific structured...... lipid (SFO), produced by enzymatic interesterification from the same oil and caprylic acid, was compared with the stability of FO. Oils were stored at 2degreesC for 11 wk followed by storage at 20degreesC for 6 wk. In addition, the antioxidative effect of adding the metal chelators EDTA or citric acid...

Amino acid and protein changes in tilapia and spanish mackerel after irradiation and storage

Energy Technology Data Exchange (ETDEWEB)

Al-Kahtani, Hassan A.; Abu-Tarboush, Hamza M.; Atia, Mohamed; Bajaber, Adnan S.; Ahmed, Mohamed A.; El-Mojaddidi, Mohamed A

1998-01-01

Some amino acids in tilapia decreased while some others increased when subjected to doses up to 10.0 kGy. However, 10 kGy contributed to a significant reduction in all amino acids of Spanish mackerel. Variations in amino acid contents continued during post-irradiation storage with no consistent trend of increase or decrease. SDS-PAGE of protein from both fish showed 27 bands of subunits with MW < 14.0-94.0 KD. Isoelectric focusing patterns of sarcoplasmic protein of unirradiated and irradiated fish showed no charge in the number of bands, while some changes were observed in the intensities of the anodic and cathodic bands depending on isoelectric points (pIs)

Modulatory Effects of Dietary Amino Acids on Neurodegenerative Diseases.

Science.gov (United States)

Rajagopal, Senthilkumar; Sangam, Supraj Raja; Singh, Shubham; Joginapally, Venkateswara Rao

2016-01-01

Proteins are playing a vital role in maintaining the cellular integrity and function, as well as for brain cells. Protein intake and supplementation of individual amino acids can affect the brain functioning and mental health, and many of the neurotransmitters in the brain are made from amino acids. The amino acid supplementation has been found to reduce symptoms, as they are converted into neurotransmitters which in turn extenuate the mental disorders. The biosynthesis of amino acids in the brain is regulated by the concentration of amino acids in plasma. The brain diseases such as depression, bipolar disorder, schizophrenia, obsessive-compulsive disorder (OCD), and Alzheimer's (AD), Parkinson's (PD), and Huntington's diseases (HD) are the most common mental disorders that are currently widespread in numerous countries. The intricate biochemical and molecular machinery contributing to the neurological disorders is still unknown, and in this chapter, we revealed the involvement of dietary amino acids on neurological diseases.

Comprehensive genetic study of fatty acids helps explain the role of noncoding inflammatory bowel disease associated SNPs and fatty acid metabolism in disease pathogenesis.

Science.gov (United States)

Jezernik, Gregor; Potočnik, Uroš

2018-03-01

Fatty acids and their derivatives play an important role in inflammation. Diet and genetics influence fatty acid profiles. Abnormalities of fatty acid profiles have been observed in inflammatory bowel diseases (IBD), a group of complex diseases defined by chronic gastrointestinal inflammation. IBD associated fatty acid profile abnormalities were observed independently of nutritional status or disease activity, suggesting a common genetic background. However, no study so far has attempted to look for overlap between IBD loci and fatty acid associated loci or investigate the genetics of fatty acid profiles in IBD. To this end, we conducted a comprehensive genetic study of fatty acid profiles in IBD using iCHIP, a custom microarray platform designed for deep sequencing of immune-mediated disease associated loci. This study identifies 10 loci associated with fatty acid profiles in IBD. The most significant associations were a locus near CBS (p = 7.62 × 10 -8 ) and a locus in LRRK2 (p = 1.4 × 10 -7 ). Of note, this study replicates the FADS gene cluster locus, previously associated with both fatty acid profiles and IBD pathogenesis. Furthermore, we identify 18 carbon chain trans-fatty acids (p = 1.12 × 10 -3 ), total trans-fatty acids (p = 4.49 × 10 -3 ), palmitic acid (p = 5.85 × 10 -3 ) and arachidonic acid (p = 8.58 × 10 -3 ) as significantly associated with IBD pathogenesis. Copyright © 2018 Elsevier Ltd. All rights reserved.

Use of deferiprone for the treatment of hepatic iron storage disease in three hornbills.

Science.gov (United States)

Sandmeier, Peter; Clauss, Marcus; Donati, Olivio F; Chiers, Koen; Kienzle, Ellen; Hatt, Jean-Michel

2012-01-01

3 hornbills (2 Papua hornbills [Aceros plicatus] and 1 longtailed hornbill [Tockus albocristatus]) were evaluated because of general listlessness and loss of feather glossiness. Because hepatic iron storage disease was suspected, liver biopsy was performed and formalin-fixed liver samples were submitted for histologic examination and quantitative image analysis (QIA). Additional frozen liver samples were submitted for chemical analysis. Birds also underwent magnetic resonance imaging (MRI) under general anesthesia for noninvasive measurement of liver iron content. Serum biochemical analysis and analysis of feed were also performed. Results of diagnostic testing indicated that all 3 hornbills were affected with hepatic iron storage disease. The iron chelator deferiprone was administered (75 mg/kg [34.1 mg/lb], PO, once daily for 90 days). During the treatment period, liver biopsy samples were obtained at regular intervals for QIA and chemical analysis of the liver iron content and follow-up MRI was performed. In all 3 hornbills, a rapid and large decrease in liver iron content was observed. All 3 methods for quantifying the liver iron content were able to verify the decrease in liver iron content. Orally administered deferiprone was found to effectively reduce the liver iron content in these 3 hornbills with iron storage disease. All 3 methods used to monitor the liver iron content (QIA, chemical analysis of liver biopsy samples, and MRI) had similar results, indicating that all of these methods should be considered for the diagnosis of iron storage disease and monitoring of liver iron content during treatment.

Muscular glycogen storage diseases without increased glycogen content on histoplathological examination

NARCIS (Netherlands)

Hoeksma, M.; den Dunnen, W. F. A.; Niezen-Koning, K. E.; van Diggelen, O. P.; van Spronsen, F. J.

Histopathological findings of muscle biopsies from five patients with two different muscular glycogen storage diseases (mGSD) were presented. From these investigations it emerged that the yield of histopathology in mGSD is low. In only one of five patients histopathological findings gave a clue

Field Sampling Plan for the HWMA/RCRA Closure Certification of the TRA-731 Caustic and Acid Storage Tank System - 1997 Notice of Violation Consent Order

International Nuclear Information System (INIS)

Evans, S.K.

2002-01-01

This Field Sampling Plan for the HWMA/RCRA Closure Certification of the TRA-731 Caustic and Acid Storage Tank System is one of two documents that comprise the Sampling and Analysis Plan for the HWMA/RCRA closure certification of the TRA-731 caustic and acid storage tank system at the Idaho National Engineering and Environmental Laboratory. This plan, which provides information about sampling design, required analyses, and sample collection and handling procedures, is to be used in conjunction with the Quality Assurance Project Plan for the HWMA/RCRA Closure Certification of the TRA-731 Caustic and Acid Storage Tank System

Preparation and characterization of stearic acid/expanded graphite composites as thermal energy storage materials

International Nuclear Information System (INIS)

Fang, Guiyin; Li, Hui; Chen, Zhi; Liu, Xu

2010-01-01

Stearic acid/expanded graphite composites with different mass ratios were prepared by absorbing liquid stearic acid into the expanded graphite. In the composite materials, the stearic acid was used as the phase change material for thermal energy storage, and the expanded graphite acted as the supporting material. Fourier transformation infrared spectroscopy, X-ray diffraction, scanning electron microscopy and thermal diffusivity measurement were used to determine the chemical structure, crystalline phase, microstructure and thermal diffusivity of the composites, respectively. The thermal properties and thermal stability were investigated by differential scanning calorimetry and thermogravimetric analysis. The thermal analysis results indicated that the materials exhibited the same phase transition characteristics as the stearic acid and their latent heats were approximately the same as the values calculated based on the weight fraction of the stearic acid in the composites. The microstructural analysis results showed that the stearic acid was well absorbed in the porous network of the expanded graphite, and there was no leakage of the stearic acid from the composites even when it was in the molten state.

Bile acids in treatment of ocular disease

OpenAIRE

Boatright, Jeffrey H.; Nickerson, John M.; Moring, Anisha G.; Pardue, Machelle T.

2009-01-01

Bear bile has been included in Asian pharmacopeias for thousands of years in treatment of several diseases, ranging from sore throat to hemorrhoids. The hydrophilic bile acids tauroursodeoxycholic acid (TUDCA) and ursodeoxycholic acid (UDCA) are the major bile acids of bear bile. Both of these are available as synthetic formulations and are approved by the health administrations of several countries for treatment of cirrhosis and gallstones. This review briefly covers the use of bear bile in ...

The antioxidant capacity of erythrocyte concentrates is increased during the first week of storage and correlated with the uric acid level.

Science.gov (United States)

Bardyn, M; Maye, S; Lesch, A; Delobel, J; Tissot, J-D; Cortés-Salazar, F; Tacchini, P; Lion, N; Girault, H H; Prudent, M

2017-10-01

Red blood cells (RBCs) suffer from lesions during cold storage, depending in part on their ability to counterbalance oxidative stress by activating their antioxidant defence. The aim of this study was to monitor the antioxidant power (AOP) in erythrocyte concentrates (ECs) during cold storage. Six ECs were prepared in saline-adenine-glucose-mannitol (SAGM) additive solution and followed during 43 days. The AOP was quantified electrochemically using disposable electrode strips and compared with results obtained from a colorimetric assay. Haematological data, data on haemolysis and the extracellular concentration of uric acid were also recorded. Additionally, a kinetic model was developed to extract quantitative kinetic data on the AOP behaviour. The AOP of total ECs and their extracellular samples attained a maximum after 1 week of storage prior to decaying and reaching a plateau, as shown by the electrochemical measurements. The observed trend was confirmed with a colorimetric assay. Uric acid had a major contribution to the extracellular AOP. Interestingly, the AOP and uric acid levels were linked to the sex of the donors. The marked increase in AOP during the first week of storage suggests that RBCs are impacted early by the modification of their environment. The AOP behaviour reflects the changes in metabolism activity following the adjustment of the extracellular uric acid level. Knowing the origin, interdonor variability and the effects of the AOP on the RBCs could be beneficial for the storage quality, which will have to be further studied. © 2017 International Society of Blood Transfusion.

Glycogen storage disease type I: clinical and laboratory profile

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Berenice L. Santos

2014-12-01

Full Text Available OBJECTIVES: To characterize the clinical, laboratory, and anthropometric profile of a sample of Brazilian patients with glycogen storage disease type I managed at an outpatient referral clinic for inborn errors of metabolism. METHODS: This was a cross-sectional outpatient study based on a convenience sampling strategy. Data on diagnosis, management, anthropometric parameters, and follow-up were assessed. RESULTS: Twenty-one patients were included (median age 10 years, range 1-25 years, all using uncooked cornstarch therapy. Median age at diagnosis was 7 months (range, 1-132 months, and 19 patients underwent liver biopsy for diagnostic confirmation. Overweight, short stature, hepatomegaly, and liver nodules were present in 16 of 21, four of 21, nine of 14, and three of 14 patients, respectively. A correlation was found between height-for-age and BMI-for-age Z-scores (r = 0.561; p = 0.008. CONCLUSIONS: Diagnosis of glycogen storage disease type I is delayed in Brazil. Most patients undergo liver biopsy for diagnostic confirmation, even though the combination of a characteristic clinical presentation and molecular methods can provide a definitive diagnosis in a less invasive manner. Obesity is a side effect of cornstarch therapy, and appears to be associated with growth in these patients.

Genetics Home Reference: glycogen storage disease type I

Science.gov (United States)

... resulting from uric acid crystals in the joints (gout), kidney disease, and high blood pressure in the ... particular ethnic groups? Genetic Changes Mutations in two genes, G6PC and SLC37A4 , cause GSDI. G6PC gene mutations ...

Survival, Quality of Life and Effects of Enzyme Replacement Therapy in Adults with Pompe Disease

OpenAIRE

Güngör, Deniz

2013-01-01

textabstractPompe disease, or glycogen storage disorder type II, is a rare inherited metabolic disorder caused by deficiency of the lysosomal enzyme acid α-glucosidase. This results in accumulation of glycogen in cells throughout the body, particularly muscle cells. The disease presents with (progressive) muscle weakness and can hence be categorized as a lysosomal storage disorder, a glycogen storage disorder and also a neuromuscular disorder. Pompe disease was the first neuromuscular disorde...

Quality Assurance Project Plan for the HWMA/RCRA Closure Certification of the TRA-731 Caustic and Acid Storage Tank System - 1997 Notice of Violation Consent Order; TOPICAL

International Nuclear Information System (INIS)

Evans, S.K.

2002-01-01

This Quality Assurance Project Plan for the HWMA/RCRA Closure Certification of the TRA- 731 Caustic and Acid Storage Tank System is one of two documents that comprise the Sampling and Analysis Plan for the HWMA/RCRA closure certification of the TRA-731 caustic and acid storage tank system at the Idaho National Engineering and Environmental Laboratory. This plan, which provides information about the project description, project organization, and quality assurance and quality control procedures, is to be used in conjunction with the Field Sampling Plan for the HWMA/RCRA Closure Certification of the TRA-731 Caustic and Acid Storage Tank System. This Quality Assurance Project Plan specifies the procedures for obtaining the data of known quality required by the closure activities for the TRA-731 caustic and acid storage tank system

Effect of Different Storage Period on Lactic Acid Bacterias from Goji Yogurt and Goji Yogurt with Honey

Directory of Open Access Journals (Sweden)

Ancuta M. Rotar

2014-05-01

Full Text Available Lactic acid bacterias (LAB: Streptococcus thermophilus-ST, Lactobacillus bulgaricus-LB are well known in the food technology area for their ability to produce lactic acid (LA from carbohydrates throught fermentation. In case of goji yogurt the interaction between the two species of LAB has influence on the fermentation period and LA quantity. LAB’s are widely used in the food industry because their growth in the dairy products lowers the carbohydrate content, they can also drop the pH values under 4.0, values were common pathogens are inhibited, and because of all these properties they are capable to prolong the shelf life. The present study aims to observe the evolution of LAB’s – ST and LB, from goji yogurt (7% (A and goji yogurt (7% with honey (B during the shelf life in corelation with other physico-chemical properties such as sugar content, fat content and dry matter. The samples for the evaluation were taken in the first day of storage, at the middle of storage period and in the last day of storage.

Role of Acid and weakly acidic reflux in gastroesophageal reflux disease off proton pump inhibitor therapy.

Science.gov (United States)

Sung, Hea Jung; Cho, Yu Kyung; Moon, Sung Jin; Kim, Jin Su; Lim, Chul Hyun; Park, Jae Myung; Lee, In Seok; Kim, Sang Woo; Choi, Myung-Gye

2012-07-01

Available data about reflux patterns and symptom determinants in the gastroesophageal reflux disease (GERD) subtypes off proton pump inhibitor (PPI) therapy are lacking. We aimed to evaluate reflux patterns and determinants of symptom perception in patients with GERD off PPI therapy by impedance-pH monitoring. We retrospectively reviewed the impedance-pH data in patients diagnosed as GERD based on results of impedance-pH monitoring, endoscopy and/or typical symptoms. The characteristics of acid and weakly acidic reflux were evaluated. Symptomatic and asymptomatic reflux were compared according to GERD subtypes and individual symptoms. Forty-two patients (22 males, mean age 46 years) were diagnosed as GERD (17 erosive reflux disease, 9 pH(+) non-erosive reflux disease [NERD], 9 hypersensitive esophagus and 7 symptomatic NERD). A total of 1,725 reflux episodes were detected (855 acid [50%], 857 weakly acidic [50%] and 13 weakly alkaline reflux [Acid reflux was more frequently symptomatic and bolus clearance was longer compared with weakly acidic reflux. In terms of globus, weakly acidic reflux was more symptomatic. Symptomatic reflux was more frequently acid and mixed reflux; these associations were more pronounced in erosive reflux disease and symptomatic NERD. The perception of regurgitation was related to acid reflux, while that of globus was more related to weakly acidic reflux. In patients not taking PPI, acid reflux was more frequently symptomatic and had longer bolus clearance. Symptomatic reflux was more frequently acid and mixed type; however, weakly acidic reflux was associated more with globus. These data suggest a role for impedance-pH data in the evaluation of globus.

Exercise in muscle glycogen storage diseases

DEFF Research Database (Denmark)

Preisler, Nicolai Rasmus; Haller, Ronald G; Vissing, John

2015-01-01

exercise program has the potential to improve general health and fitness and improve quality of life, if executed properly. In this review, we describe skeletal muscle substrate use during exercise in GSDs, and how blocks in metabolic pathways affect exercise tolerance in GSDs. We review the studies...... that have examined the effect of regular exercise training in different types of GSD. Finally, we consider how oral substrate supplementation can improve exercise tolerance and we discuss the precautions that apply to persons with GSD that engage in exercise.......Glycogen storage diseases (GSD) are inborn errors of glycogen or glucose metabolism. In the GSDs that affect muscle, the consequence of a block in skeletal muscle glycogen breakdown or glucose use, is an impairment of muscular performance and exercise intolerance, owing to 1) an increase...

Characteristics of Oxidative Storage Stability of Canola Fatty Acid Methyl Ester Stabilised with Antioxidants

Directory of Open Access Journals (Sweden)

Tirto Prakoso

2012-11-01

Full Text Available The storage effects on the oxidation characteristics of fatty acid methyl ester of canola oil (CME were investigated in this study. CME stabilised with two antioxidants, i.e. 2,6-di-tert-bytyl-p-cresol (BHT and 6,6-di-tert-butyl-2, 2’-methylendi-p-cresol (BPH, was stored at 20, 40 and 60°C. The oxidation stability data were measured by the Rancimat test method and it was found that both BHT and BPH addition increased the oxidation resistance of the CME. The results showed that when BPH or BHT was added at a concentration of 100 ppm, the oxidation induction period of the neat CME samples increased from 5.53 h to 6.93 h and 6.14 h, respectively. Comparing both antioxidants, BPH proved to be more effective in increasing the oxidation resistance when both antioxidants were added at the same concentration. Furthermore, the oxidation induction time decreased linearly with the storage time. It was shown that the oxidation occurred rapidly in the first 8 weeks of storage. Later, a kinetic study was undertaken and first-order kinetics were applied to explain the oxidation characteristics of the CME added with antioxidants. This kinetic study focused on exploiting the activation energy values obtained from the Arrhenius equations. Also, the oxidation effects on other quality parameters, including acid value, peroxide value, kinematic viscosity, and water content, were examined.

Role of Acid and Weakly Acidic Reflux in Gastroesophageal Reflux Disease Off Proton Pump Inhibitor Therapy

Science.gov (United States)

Sung, Hea Jung; Moon, Sung Jin; Kim, Jin Su; Lim, Chul Hyun; Park, Jae Myung; Lee, In Seok; Kim, Sang Woo; Choi, Myung-Gye

2012-01-01

Background/Aims Available data about reflux patterns and symptom determinants in the gastroesophageal reflux disease (GERD) subtypes off proton pump inhibitor (PPI) therapy are lacking. We aimed to evaluate reflux patterns and determinants of symptom perception in patients with GERD off PPI therapy by impedance-pH monitoring. Methods We retrospectively reviewed the impedance-pH data in patients diagnosed as GERD based on results of impedance-pH monitoring, endoscopy and/or typical symptoms. The characteristics of acid and weakly acidic reflux were evaluated. Symptomatic and asymptomatic reflux were compared according to GERD subtypes and individual symptoms. Results Forty-two patients (22 males, mean age 46 years) were diagnosed as GERD (17 erosive reflux disease, 9 pH(+) non-erosive reflux disease [NERD], 9 hypersensitive esophagus and 7 symptomatic NERD). A total of 1,725 reflux episodes were detected (855 acid [50%], 857 weakly acidic [50%] and 13 weakly alkaline reflux [reflux was more frequently symptomatic and bolus clearance was longer compared with weakly acidic reflux. In terms of globus, weakly acidic reflux was more symptomatic. Symptomatic reflux was more frequently acid and mixed reflux; these associations were more pronounced in erosive reflux disease and symptomatic NERD. The perception of regurgitation was related to acid reflux, while that of globus was more related to weakly acidic reflux. Conclusions In patients not taking PPI, acid reflux was more frequently symptomatic and had longer bolus clearance. Symptomatic reflux was more frequently acid and mixed type; however, weakly acidic reflux was associated more with globus. These data suggest a role for impedance-pH data in the evaluation of globus. PMID:22837877

Combining nutrition, food science and engineering in developing solutions to Inflammatory bowel diseases--omega-3 polyunsaturated fatty acids as an example.

Science.gov (United States)

Ferguson, Lynnette R; Smith, Bronwen G; James, Bryony J

2010-10-01

The Inflammatory bowel diseases, Crohn's disease and ulcerative colitis, are debilitating conditions, characterised by lifelong sensitivity to certain foods, and often a need for surgery and life-long medication. The anti-inflammatory effects of long chain omega-3 polyunsaturated acids justify their inclusion in enteral nutrition formulas that have been associated with disease remission. However, there have been variable data in clinical trials to test supplementary omega-3 polyunsaturated fatty acids in inducing or maintaining remission in these diseases. Although variability in trial design has been suggested as a major factor, we suggest that variability in processing and presentation of the products may be equally or more important. The nature of the source, and rapidity of getting the fish or other food source to processing or to market, will affect the percentage of the various fatty acids, possible presence of heavy metal contaminants and oxidation status of the various fatty acids. For dietary supplements or fortified foods, whether the product is encapsulated or not, whether storage is under nitrogen or not, and length of time between harvest, processing and marketing will again profoundly affect the properties of the final product. Clinical trials to test efficacy of these products in IBD to date have utilised the relevant skills of pharmacology and gastroenterology. We suggest that knowledge from food science, nutrition and engineering will be essential to establish the true role of this important group of compounds in these diseases. This journal is © The Royal Society of Chemistry 2010

Folic acid and diseases - supplement it or not?

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Siaw-Cheok Liew

2016-02-01

Full Text Available SUMMARY Introduction: folic acid is a water soluble vitamin, which is synthetically-produced and found in fortified foods and supplements. Folate is found naturally in plants, such as the dark green leafy vegetables. Folate is not synthesizedde novo by humans, therefore the daily requirements are met from the dietary intake of folic acid supplements or food rich in this vitamin. Folate deficiency could lead to numerous common health problems. Hyperhomocysteinemia and the possibility of malignancy developments are the long term consequences of this deficit albeit contradictory findings on these claims. Methods: the articles included in this review focused on recent updated evidence-based reports and meta-analyses on the associations of the serum folate/folic acid and the various diseases found globally. Results: the benefit of folic acid supplementation in the pre-conception period for the prevention of neural tube defects (NTDs was well established and it was suggested that counseling sessions should be given to women with previous pregnancies affected by NTDs. However, supplementation of folic acid and its medicinal effects in the treatment of other diseases were contradictory and unclear. Conclusion: more detailed investigations into the health benefits of folic acid are needed before it could be recommended for supplementation, treatment or prevention of some of the diseases discussed in this review.

The acid perfusion test in gastroesophageal reflux disease

International Nuclear Information System (INIS)

Kaul, B.; Petersen, H.; Grette, K.; Myrvold, H.E.

1986-01-01

An acid perfusion test, isotope scanning, endoscopy, and esophageal biopsy were performed in 101 patients with symptoms strongly suggestive of gastroesophageal reflux (GER) disease. A positive acid perfusion test within 30 min (APT) and within 5 min (TAPT) was found in 70.2% and 37.6% of the patients, respectively. A positive APT was found significantly more often in patients with than without endoscopic esophagitis, whereas a positive TAPT was found significantly more often in patients with severe symptoms than in patients with moderate symptoms, and in a significantly higher proportion of patients with than without GER by scintigraphy. Neither the APT nor the TAPT showed any dependency on the presence of histologic esophagitis. Most (97%) patients with a negative acid perfusion test, in addition to typical symptoms, also presented with scintigraphic, endoscopic, or histologic evidence of GER disease. Although it shows that the acid perfusion test, particularly when early positive, may serve as a weak predictor of the severity of GER disease, the present study gives little support to the test's clinical usefulness

Changes in some physicochemical properties and fatty acid composition of irradiated meatballs during storage

Energy Technology Data Exchange (ETDEWEB)

Gecgel, U., E-mail: ugecgel@nku.edu.tr [Agricultural Faculty, Department of Food Engineering, Namik Kemal University, 59030 Tekirdag (Turkey)

2013-05-15

Meatball samples were irradiated using a {sup 60}Co irradiation source (with the dose of 1, 3, 5 and 7 kGy) and stored (1, 2 and 3 weeks at 4 Degree-Sign C) to appraise some physicochemical properties and the fatty acid composition. The physicochemical results showed no significant differences in moisture, protein, fat and ash content of meatballs because of irradiation. However, total acidity, peroxide and thiobarbituric acid (TBA) values increased significantly as a result of irradiation doses and storage period. The fatty acid profile in meatball samples changed with irradiation. While saturated fatty acids (C16:0, C17:0, C18:0, and C20:0) increased with irradiation, monounsaturated (C14:1, C15:1, C18:1, and C20:1) and polyunsaturated (C18:2, C18:3, and C22:2) fatty acids decreased with irradiation. Trans fatty acids (C16:1trans, C18:1trans, C18:2trans, C18:3trans) increased with increasing irradiation doses. Meatball samples irradiated at 7 kGy had the highest total trans fatty acid content. This research shows that some physicochemical properties and fatty acid composition of meatballs can be changed by gamma irradiation. (author)

Changes in some physicochemical properties and fatty acid composition of irradiated meatballs during storage.

Science.gov (United States)

Gecgel, Umit

2013-06-01

Meatball samples were irradiated using a (60)Co irradiation source (with the dose of 1, 3, 5 and 7 kGy) and stored (1, 2 and 3 weeks at 4°C) to appraise some physicochemical properties and the fatty acid composition. The physicochemical results showed no significant differences in moisture, protein, fat and ash content of meatballs because of irradiation. However, total acidity, peroxide and thiobarbituric acid (TBA) values increased significantly as a result of irradiation doses and storage period. The fatty acid profile in meatball samples changed with irradiation. While saturated fatty acids (C16:0, C17:0, C18:0, and C20:0) increased with irradiation, monounsaturated (C14:1, C15:1, C18:1, and C20:1) and polyunsaturated (C18:2, C18:3, and C22:2) fatty acids decreased with irradiation. Trans fatty acids (C16:1trans, C18:1trans, C18:2trans, C18:3trans) increased with increasing irradiation doses. Meatball samples irradiated at 7 kGy had the highest total trans fatty acid content. This research shows that some physicochemical properties and fatty acid composition of meatballs can be changed by gamma irradiation.

Changes in some physicochemical properties and fatty acid composition of irradiated meatballs during storage

International Nuclear Information System (INIS)

Gecgel, U.

2013-01-01

Meatball samples were irradiated using a 60 Co irradiation source (with the dose of 1, 3, 5 and 7 kGy) and stored (1, 2 and 3 weeks at 4 °C) to appraise some physicochemical properties and the fatty acid composition. The physicochemical results showed no significant differences in moisture, protein, fat and ash content of meatballs because of irradiation. However, total acidity, peroxide and thiobarbituric acid (TBA) values increased significantly as a result of irradiation doses and storage period. The fatty acid profile in meatball samples changed with irradiation. While saturated fatty acids (C16:0, C17:0, C18:0, and C20:0) increased with irradiation, monounsaturated (C14:1, C15:1, C18:1, and C20:1) and polyunsaturated (C18:2, C18:3, and C22:2) fatty acids decreased with irradiation. Trans fatty acids (C16:1trans, C18:1trans, C18:2trans, C18:3trans) increased with increasing irradiation doses. Meatball samples irradiated at 7 kGy had the highest total trans fatty acid content. This research shows that some physicochemical properties and fatty acid composition of meatballs can be changed by gamma irradiation. (author)

PPAR/RXR Regulation of Fatty Acid Metabolism and Fatty Acid -Hydroxylase (CYP4 Isozymes: Implications for Prevention of Lipotoxicity in Fatty Liver Disease

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James P. Hardwick

2009-01-01

Full Text Available Fatty liver disease is a common lipid metabolism disorder influenced by the combination of individual genetic makeup, drug exposure, and life-style choices that are frequently associated with metabolic syndrome, which encompasses obesity, dyslipidemia, hypertension, hypertriglyceridemia, and insulin resistant diabetes. Common to obesity related dyslipidemia is the excessive storage of hepatic fatty acids (steatosis, due to a decrease in mitochondria -oxidation with an increase in both peroxisomal -oxidation, and microsomal -oxidation of fatty acids through peroxisome proliferator activated receptors (PPARs. How steatosis increases PPAR activated gene expression of fatty acid transport proteins, peroxisomal and mitochondrial fatty acid -oxidation and -oxidation of fatty acids genes regardless of whether dietary fatty acids are polyunsaturated (PUFA, monounsaturated (MUFA, or saturated (SFA may be determined by the interplay of PPARs and HNF4 with the fatty acid transport proteins L-FABP and ACBP. In hepatic steatosis and steatohepatitis, the -oxidation cytochrome P450 CYP4A gene expression is increased even with reduced hepatic levels of PPAR. Although numerous studies have suggested the role ethanol-inducible CYP2E1 in contributing to increased oxidative stress, Cyp2e1-null mice still develop steatohepatitis with a dramatic increase in CYP4A gene expression. This strongly implies that CYP4A fatty acid -hydroxylase P450s may play an important role in the development of steatohepatitis. In this review and tutorial, we briefly describe how fatty acids are partitioned by fatty acid transport proteins to either anabolic or catabolic pathways regulated by PPARs, and we explore how medium-chain fatty acid (MCFA CYP4A and long-chain fatty acid (LCFA CYP4F -hydroxylase genes are regulated in fatty liver. We finally propose a hypothesis that increased CYP4A expression with a decrease in CYP4F genes may promote the progression of steatosis to

ACID VALUE OF VEGETABLE OILS AND POULTRY FEED AS AFECTED BY STORAGE PERIOD AND ANTIOXIDANTS

Directory of Open Access Journals (Sweden)

Sohail Hassan Khan, Bashir Mahmood Bhatti and Rozina Sardar

2001-09-01

Full Text Available A study to assess acid values in soyabean, cotton seed and sunflower oil commonly used in poultry ration was conducted. It was observed that mean acid value of oils ~ept in open were significantly high (7.67 than oil kept in sealed form (1.296. The mean acid value was higher in soyabean oil (P<0.01 than the values in cotton seed oil and sunflower oil. While determining the effect of Santaquin, BHT and Oxistat as antioxidant, in the ration stored at 40 °C for 2 months, it was observed that the acid values in untreated control ration was 18.20 while with the added antioxidants were 4.88, 4.85 and 4.83, respectively showing a significant increase with each week of the storage.

Effects of brown seaweed polyphenols, α-tocopherol, and ascorbic acid on protein oxidation and textural properties of fish mince (Pagrosomus major) during frozen storage.

Science.gov (United States)

Wang, Tiantian; Li, Zhenxing; Yuan, Fangzhou; Lin, Hong; Pavase, Tushar Ramesh

2017-03-01

Frozen storage of minced fish is currently one of the most important techniques to maintain its functional properties. However, some deterioration does occur during frozen storage and cause quality loss. The effects of brown seaweed polyphenols, α-tocopherol, and ascorbic acid on lipid and protein oxidation and textural properties of red sea bream (Pagrosomus major) during 90 days of frozen storage at -18 °C were investigated. All added antioxidants at 1 g kg -1 resulted in significantly lower thiobarbituric acid-reactive substances (TBARS) compared to the control during the 45 days of frozen storage. The antioxidants were also effective in retarding protein oxidation concerning to total sulfhydryl content and protein carbonyl content. Brown seaweed polyphenols and α-tocopherol significantly retarded the inactivation of Ca 2+ -ATPase activity during the first 45 days, whereas ascorbic acid had no such effect. The antioxidant activity showed either an invariable or decrease trend after 45 days storage. Adding antioxidants had a significant effect on the breaking force of the gels during the frozen storage period. These results indicate that brown seaweed polyphenols and α-tocopherol can be used to prevent oxidative reactions and thus maintain the structure of the gel formed by fish mince during frozen storage. © 2016 Society of Chemical Industry. © 2016 Society of Chemical Industry.

Uric acid and chronic kidney disease: which is chasing which?

Science.gov (United States)

Johnson, Richard J.; Nakagawa, Takahiko; Jalal, Diana; Sánchez-Lozada, Laura Gabriela; Kang, Duk-Hee; Ritz, Eberhard

2013-01-01

Serum uric acid is commonly elevated in subjects with chronic kidney disease (CKD), but was historically viewed as an issue of limited interest. Recently, uric acid has been resurrected as a potential contributory risk factor in the development and progression of CKD. Most studies documented that an elevated serum uric acid level independently predicts the development of CKD. Raising the uric acid level in rats can induce glomerular hypertension and renal disease as noted by the development of arteriolosclerosis, glomerular injury and tubulointerstitial fibrosis. Pilot studies suggest that lowering plasma uric acid concentrations may slow the progression of renal disease in subjects with CKD. While further clinical trials are necessary, uric acid is emerging as a potentially modifiable risk factor for CKD. Gout was considered a cause of CKD in the mid-nineteenth century [1], and, prior to the availability of therapies to lower the uric acid level, the development of end-stage renal disease was common in gouty patients. In their large series of gouty subjects Talbott and Terplan found that nearly 100% had variable degrees of CKD at autopsy (arteriolosclerosis, glomerulosclerosis and interstitial fibrosis) [2]. Additional studies showed that during life impaired renal function occurred in half of these subjects [3]. As many of these subjects had urate crystals in their tubules and interstitium, especially in the outer renal medulla, the disease became known as gouty nephropathy. The identity of this condition fell in question as the presence of these crystals may occur in subjects without renal disease; furthermore, the focal location of the crystals could not explain the diffuse renal scarring present. In addition, many subjects with gout also had coexistent conditions such as hypertension and vascular disease, leading some experts to suggest that the renal injury in gout was secondary to these latter conditions rather than to uric acid per se [4]. Indeed, gout was

Prediction of the ageing of commercial lager beer during storage based on the degradation of iso-α-acids.

Science.gov (United States)

Blanco, Carlos A; Nimubona, Dieudonné; Caballero, Isabel

2014-08-01

Iso-α-acids and their chemically modified variants are responsible for the bitterness of beer and play a disproportionately large role in the final quality of beer. The current study was undertaken to predict the degradation of commercial lager beers related to changes in the concentration of trans-iso-α-acids during storage by using high-pressure liquid chromatography. In the analysed beers the concentration of isohumulone (average concentration 28 mg L(-1)) was greater than that of isocohumulone (20 mg L(-1)) and isoadhumulone (10 mg L(-1)). The kinetic parameters, activation energy and rate constant, of the trans-iso-α-acids were calculated. In the case of dark beers, the activation energy for the degradation of trans-isocohumulones was found to be higher than for trans-isohumulones and trans-isoadhumulones, whereas in pale and alcohol-free beers activation energies for the degradation of the three trans isomers were similar. The loss of iso-α-acids can be calculated using the activation energy of the degradation of trans-iso-α-acids and the temperature profile of the accelerated ageing. The results obtained in the investigation can be used in the beer industry to predict the alteration of the bitterness of beer during storage. © 2013 Society of Chemical Industry.

Influence of chain length of pyrene fatty acids on their uptake and metabolism by Epstein-Barr-virus-transformed lymphoid cell lines from a patient with multisystemic lipid storage myopathy and from control subjects.

Science.gov (United States)

Radom, J; Salvayre, R; Levade, T; Douste-Blazy, L

1990-01-01

The uptake and intracellular metabolism of 4-(1-pyrene)butanoic acid (P4), 10-(1-pyrene)decanoic acid (P10) and 12-(1-pyrene)dodecanoic acid (P12) were investigated in cultured lymphoid cell lines from normal individuals and from a patient with multisystemic lipid storage myopathy (MLSM). The cellular uptake was shown to be dependent on the fatty-acid chain length, but no significant difference in the uptake of pyrene fatty acids was observed between MLSM and control lymphoid cells. After incubation for 1 h the distribution of fluorescent fatty acids taken up by the lymphoid cell lines also differed with the chain length, most of the fluorescence being associated with phospholipid and triacylglycerols. In contrast with P10 and P12, P4 was not incorporated into neutral lipids. When the cells were incubated for 24 h with the pyrene fatty acids, the amount of fluorescent lipids synthesized by the cells was proportional to the fatty acid concentration in the culture medium. After a 24 h incubation in the presence of P10 or P12, at any concentration, the fluorescent triacylglycerol content of MLSM cells was 2-5-fold higher than that of control cells. Concentrations of pyrene fatty acids higher than 40 microM seemed to be more toxic for mutant cells than for control cells. This cytotoxicity was dependent on the fluorescent-fatty-acid chain length (P12 greater than P10 greater than P4). Pulse-chase experiments permitted one to demonstrate the defect in the degradation of endogenously biosynthesized triacylglycerols in MLSM cells (residual activity was around 10-25% of controls on the basis of half-lives and initial rates of P10- or P12-labelled-triacylglycerol catabolism); MLSM lymphoid cells exhibited a mild phenotypic expression of the lipid storage (less severe than that observed in fibroblasts). P4 was not utilized in the synthesis of triacylglycerols, and thus did not accumulate in MLSM cells: this suggests that natural short-chain fatty acids might induce a lesser

Diagnosis of Pompe disease

DEFF Research Database (Denmark)

Vissing, John; Lukacs, Zoltan; Straub, Volker

2013-01-01

The diagnosis of Pompe disease (acid maltase deficiency, glycogen storage disease type II) in children and adults can be challenging because of the heterogeneous clinical presentation and considerable overlap of signs and symptoms found in other neuromuscular diseases. This review evaluates some...... to identify late-onset Pompe disease often leads to false-negative results and subsequent delays in identification and treatment of the disorder. Serum creatine kinase level can be normal or only mildly elevated in late-onset Pompe disease and is not very helpful alone to suggest the diagnosis...

Energy storage

Science.gov (United States)

Kaier, U.

1981-04-01

Developments in the area of energy storage are characterized, with respect to theory and laboratory, by an emergence of novel concepts and technologies for storing electric energy and heat. However, there are no new commercial devices on the market. New storage batteries as basis for a wider introduction of electric cars, and latent heat storage devices, as an aid for solar technology applications, with satisfactory performance standards are not yet commercially available. Devices for the intermediate storage of electric energy for solar electric-energy systems, and for satisfying peak-load current demands in the case of public utility companies are considered. In spite of many promising novel developments, there is yet no practical alternative to the lead-acid storage battery. Attention is given to central heat storage for systems transporting heat energy, small-scale heat storage installations, and large-scale technical energy-storage systems.

Opposed-phase MR imaging of lipid storage myopathy in a case of Chanarin-Dorfman disease

International Nuclear Information System (INIS)

Gaeta, Michele; Celona, Antonio; Racchiusa, Sergio; Mazziotti, Silvio; Minutoli, Fabio; Toscano, Antonio; Musumeci, Olimpia

2008-01-01

Chanarin-Dorfman disease (CDD) is a rare genetic disorder characterized by ichthyosis, myopathy, central nervous system disturbances, and intracellular lipid storage in muscle fibers, hepatocytes, and granulocytes. We describe skeletal muscle magnetic resonance imaging findings in a case of CDD, outlining the potential role of GE T1-weighted opposed-phase sequence (chemical shift imaging) in the evaluation of lipid storage myopathies. (orig.)

Opposed-phase MR imaging of lipid storage myopathy in a case of Chanarin-Dorfman disease

Energy Technology Data Exchange (ETDEWEB)

Gaeta, Michele; Celona, Antonio; Racchiusa, Sergio; Mazziotti, Silvio [University of Messina, Department of Radiological Sciences, Messina (Italy); Minutoli, Fabio [University of Messina, Department of Radiological Sciences, Messina (Italy); A.O.U. ' ' Policlinico G. Martino' ' , Dipartimento di Scienze Radiologiche, Messina (Italy); Toscano, Antonio; Musumeci, Olimpia [University of Messina, Department of Neurosciences, Psychiatry and Anaesthesiology, Messina (Italy)

2008-11-15

Chanarin-Dorfman disease (CDD) is a rare genetic disorder characterized by ichthyosis, myopathy, central nervous system disturbances, and intracellular lipid storage in muscle fibers, hepatocytes, and granulocytes. We describe skeletal muscle magnetic resonance imaging findings in a case of CDD, outlining the potential role of GE T1-weighted opposed-phase sequence (chemical shift imaging) in the evaluation of lipid storage myopathies. (orig.)

Zoledronic acid in metastatic bone disease: an audit based discussion

International Nuclear Information System (INIS)

Akbar, R.A.; Gosh, S.

2010-01-01

Background: Metastatic bone disease is a common problem in patients with advanced cancer causing significant morbidity and poor quality of life. Effective and less toxic treatments, like bisphophonates, can reduce morbidity in such cases. Objectives: The objectives of this study were to determine whether Zoledronic acid was administered in accordance with current recommendations for its prescribing and to produce protocols for improved patient outcomes. Methods: The study was a retrospective audit of 39 consecutive patients with metastatic bone disease secondary to solid tumours who were treated with Zoledronic acid. The records were analysed to establish the administered dose of Zoledronic acid relative to creatinine clearance. The standards for Zoledronic acid therapy were defined from best practice guidelines. Results: The commonest diagnosis in patients receiving Zoledronic acid was carcinoma prostate 19/39 (49%) followed by carcinoma breast 11/39 (28%), gastrointestinal malignancies 4/39 (10%) and renal cell carcinoma 3/39 (8%). Indications for therapy were metastatic bone disease alone 31 (79%), hypercalcaemia alone 0/39 (0%), metastatic bone disease with hypercalcaemia 5/39 (13%), and prevention of chemotherapy induced bone loss 1/39 (3%). The dose of Zoledronic acid was appropriate to the creatinine clearance in 25/39 (6 4%), inappropriate in 5/39 (13%) and unclear from the notes in 9/39 (23%). Conclusions: Majority of patients received Zoledronic acid for the appropriate indications. The dose of Zoledronic acid was appropriate to serum creatinine clearance in a majority of patients. Poor documentation of data pertaining to Zoledronic acid treatment is observed which can potentially lead to major errors in prescribing. We recommend using a standard form to document each episode of therapy with Zoledronic acid. (author)

A patient with common glycogen storage disease type Ib mutations without neutropenia or neutrophil dysfunction

NARCIS (Netherlands)

Martens, DHJ; Kuijpers, TW; Maianski, NA; Rake, JP; Smit, GPA; Visser, G

We describe a 16-year old boy with glycogen storage disease type Ib, homozygous for the common 1211-1212delCT mutation, who never experienced neutropenia, and did not suffer from frequent infections or inflammatory bowel disease. In addition, neutrophil function tests showed no abnormalities.

Marine OMEGA-3 fatty acids in the prevention of cardiovascular disease.

Science.gov (United States)

Mori, Trevor A

2017-11-01

Omega-6 (ω6) and omega-3 (ω3) fatty acids are two classes of dietary polyunsaturated fatty acids derived from linoleic acid (18:2ω6) and α-linolenic acid (18:3ω3), respectively. Enzymatic metabolism of linoleic and α-linolenic acids generates arachidonic acid (20:4ω6) and eicosapentaenoic acid (20:5ω3; EPA), respectively, both of which are substrates for enzymes that yield eicosanoids with multiple and varying physiological functions. Further elongation and desaturation of EPA yields the 22-carbon fatty acid docosahexaenoic acid (22:6ω3; DHA). The main dietary source of EPA and DHA for human consumption is fish, especially oily fish. There is considerable evidence that EPA and DHA are protective against cardiovascular disease (heart disease and stroke), particularly in individuals with pre-existing disease. ω3 Fatty acids benefit multiple risk factors including blood pressure, blood vessel function, heart function and blood lipids, and they have antithrombotic, anti-inflammatory and anti-oxidative actions. ω3 Fatty acids do not adversely interact with medications. Supplementation with ω3 fatty acids is recommended in individuals with elevated blood triglyceride levels and patients with coronary heart disease. A practical recommendation for the general population is to increase ω3 fatty acid intake by incorporating fish as part of a healthy diet that includes increased fruits and vegetables, and moderation of salt intake. Health authorities recommend the general population should consume at least two oily fish meals per week. Copyright © 2017 Elsevier B.V. All rights reserved.

Therapeutic Mechanisms of Bile Acids and Nor-Ursodeoxycholic Acid in Non-Alcoholic Fatty Liver Disease.

Science.gov (United States)

Steinacher, Daniel; Claudel, Thierry; Trauner, Michael

2017-01-01

Non-alcoholic fatty liver disease is one of the most rapidly rising clinical problems in the 21st century. So far no effective drug treatment has been established to cure this disease. Bile acids (BAs) have a variety of signaling properties, which can be used therapeutically for modulating hepatic metabolism and inflammation. A side-chain shorted derivative of ursodeoxycholic acid (UDCA) is 24 nor-ursodeoxycholic acid (NorUDCA) and it represents a new class of drugs for treatment of liver diseases. NorUDCA has unique biochemical and therapeutic properties, since it is relatively resistant to conjugation with glycine or taurine compared to UDCA. NorUDCA undergoes cholehepatic shunting, resulting in ductular targeting, bicarbonate-rich hypercholeresis, and cholangiocyte protection. Furthermore, it showed anti-fibrotic, anti-inflammatory, and anti-lipotoxic properties in several animal models. As such, NorUDCA is a promising new approach in the treatment of cholestatic and metabolic liver diseases. This review is a summary of current BA-based therapeutic approaches in the treatment of the fatty liver disease. © 2017 S. Karger AG, Basel.

Adiponectin levels correlate with the severity of hypertriglyceridaemia in glycogen storage disease Ia

NARCIS (Netherlands)

Bandsma, R. H. J.; Smit, G. P. A.; Reijngoud, D. -J.; Kuipers, F.

2009-01-01

Glycogen storage disease type Ia (GSD Ia) is characterized by severe hypercholesterolaemia and hypertriglyceridaemia. Little is known about the aetiology of the hyperlipidaemia in GSD Ia. Adipokines play an important regulatory role in lipid metabolism. We investigated whether adipokine

Effects of Formic or Acetic Acid on the Storage Quality of Mixed Air-Dried Corn Stover and Cabbage Waste, and Microbial Community Analysis

Directory of Open Access Journals (Sweden)

Cong Wang

2018-01-01

Full Text Available A mixture of air-dried corn stover and cabbage waste was ensiled to preserve lignocellulosic biomass for use as biofuel. Furthermore, the effects of different fresh mass fractions (0.3 and 0.6 % of formic or acetic acid on the mixed silage quality were evaluated to guarantee its quality. The application of formic or acetic acid prior to mixing the silage led to higher water-soluble carbohydrate fractions than the negative control, indicating that both acids contributed to preservation of water-soluble carbohydrates during storage for 170 days. The dry matter content was also increased after storage from 90 to 170 days. It was found that the content of neutral and acid detergent fibre, cellulose and holocellulose (the sum of cellulose and hemicellulose in mixed silage treated with formic or acetic acid was significantly lower than that obtained in the negative control. The pH and the ratio of ammoniacal nitrogen to total nitrogen in mixed silage treated with acetic acid also significantly decreased. Furthermore, the addition of formic or acetic acid significantly weakened the fermentation intensity of lactic acid, depending on the ratio of lactic to acetic acid, as well as the ratio of lactic acid to total organic acids. The number of bacterial species and their relative abundance shifted during silage mixing, wherein microbial communities at phylum level mainly consisted of Proteobacteria and Firmicutes. The dominant bacteria were also observed to shift from Lactobacillus and Enterobacter in presilage biomass to Lactobacillus and Paralactobacillus. Specifically, Enterobacter disappeared after 130 days of storage. In conclusion, the addition of a low dose of acetic acid to fresh mass (0.3 % could effectively improve the fermentation quality and is conducive to the preservation of the organic components.

Understanding the degradation of ascorbic acid and glutathione in relation to the levels of oxidative stress biomarkers in broccoli (Brassica oleracea L. italica cv. Bellstar) during storage and mechanical processing.

Science.gov (United States)

Raseetha, Siva; Leong, Sze Ying; Burritt, David John; Oey, Indrawati

2013-06-01

The purpose of this research was to understand the degradation of ascorbic acid and glutathione content in broccoli florets (Brassica oleracea L. italica cv. Bellstar) during prolonged storage and subsequent mechanical processing. The initial content of total ascorbic acid and glutathione in broccoli florets averaged at 5.18 ± 0.23 and 0.70 ± 0.03 μmol/g fresh weight, respectively. Results showed that the content of ascorbic acid and glutathione in broccoli degraded during storage at 23°C, for at least 4.5-fold after 6 days of storage. On each day of storage, broccoli florets were mechanically processed, but the content of total ascorbic acid and glutathione was not significantly affected. When the mechanically processed broccoli florets were further incubated for up to 6h, the amount of ascorbic acid was greatly reduced as compared to glutathione. To obtain an in-depth understanding on the degradation of ascorbic acid and glutathione, the activity of enzymes involved in plant antioxidative system via ascorbate-glutathione cycle, as a response towards oxidative stress that took place during storage was determined in this study. The content of total ascorbic acid and glutathione in broccoli florets before and after mechanical processing were found to decrease concurrently with the activity of ascorbic acid peroxidase and glutathione reductase over the experimental storage duration. Meanwhile, the effect of oxidative stress on the content of ascorbic acid and glutathione was apparent during the 6h of incubation after mechanical processing. This phenomenon was demonstrated by the level of oxidative stress biomarkers examined, in which the formation of lipid peroxides, protein carbonyls and DNA oxidised products was positively associated with the degradation of total ascorbic acid and glutathione. Copyright © 2012 Elsevier Ltd. All rights reserved.

The National Institute on Aging Genetics of Alzheimer’s Disease Data Storage Site (NIAGADS)

Data.gov (United States)

U.S. Department of Health & Human Services — The National Institute on Aging Genetics of Alzheimer's Disease Data Storage Site (NIAGADS) is a national genetics data repository facilitating access to genotypic...

Impaired Autophagy in the Lipid-Storage Disorder Niemann-Pick Type C1 Disease

OpenAIRE

Sarkar, Sovan; Carroll, Bernadette; Buganim, Yosef; Maetzel, Dorothea; Ng, Alex H.M.; Cassady, John P.; Cohen, Malkiel A.; Chakraborty, Souvik; Wang, Haoyi; Spooner, Eric; Ploegh, Hidde; Gsponer, Joerg; Korolchuk, Viktor I.; Jaenisch, Rudolf

2013-01-01

Autophagy dysfunction has been implicated in misfolded protein accumulation and cellular toxicity in several diseases. Whether alterations in autophagy also contribute to the pathology of lipid-storage disorders is not clear. Here, we show defective autophagy in Niemann-Pick type C1 (NPC1) disease associated with cholesterol accumulation, where the maturation of autophagosomes is impaired because of defective amphisome formation caused by failure in SNARE machinery, whereas the lysosomal prot...

Type IIIb glycogen storage disease associated with end-stage cirrhosis and hepatocellular carcinoma

NARCIS (Netherlands)

Haagsma, EB; Smit, GPA; NiezenKoning, KE; Gouw, ASH; Meerman, L; Slooff, MJH

Type III glycogen storage disease (GSD) is a disorder of carbohydrate metabolism caused by a deficiency of debranching enzyme. Different subtypes with different clinical pictures have been recognized. During childhood and early adulthood, the symptoms generally regress, and normal adulthood appears

Common and uncommon pathogenic cascades in lysosomal storage diseases.

Science.gov (United States)

Vitner, Einat B; Platt, Frances M; Futerman, Anthony H

2010-07-02

Lysosomal storage diseases (LSDs), of which about 50 are known, are caused by the defective activity of lysosomal proteins, resulting in accumulation of unmetabolized substrates. As a result, a variety of pathogenic cascades are activated such as altered calcium homeostasis, oxidative stress, inflammation, altered lipid trafficking, autophagy, endoplasmic reticulum stress, and autoimmune responses. Some of these pathways are common to many LSDs, whereas others are only altered in a subset of LSDs. We now review how these cascades impact upon LSD pathology and suggest how intervention in the pathways may lead to novel therapeutic approaches.

A NOVEL PNYSIOLOGICALLY BASED PHARMACOKINETIC (PBPK) MODEL FOR DIMETHYLARSINIC ACID (DMA): THE LUNG AS A STORAGE COMPARTMENT

Science.gov (United States)

A NOVEL PHYSIOLOGICALLY-BASED PHARMACOKINETIC (PBPK) MODEL FOR DIMETHYLARSINIC ACID (DMA): THE LUNG AS A STORAGE COMPARTMENT. Evans, M.V., Hughes, M.F., and Kenyon, E.M. USEPA, ORD, NHEERL, RTP, NC 27711DMA is the major methylated metabolite of inorganic arsenic, a kno...

Rapid screening for lipid storage disorders using biochemical markers. Expert center data and review of the literature.

Science.gov (United States)

Voorink-Moret, M; Goorden, S M I; van Kuilenburg, A B P; Wijburg, F A; Ghauharali-van der Vlugt, J M M; Beers-Stet, F S; Zoetekouw, A; Kulik, W; Hollak, C E M; Vaz, F M

2018-02-01

In patients suspected of a lipid storage disorder (sphingolipidoses, lipidoses), confirmation of the diagnosis relies predominantly on the measurement of specific enzymatic activities and genetic studies. New UPLC-MS/MS methods have been developed to measure lysosphingolipids and oxysterols, which, combined with chitotriosidase activity may represent a rapid first tier screening for lipid storage disorders. A lysosphingolipid panel consisting of lysoglobotriaosylceramide (LysoGb3), lysohexosylceramide (LysoHexCer: both lysoglucosylceramide and lysogalactosylceramide), lysosphingomyelin (LysoSM) and its carboxylated analogue lysosphingomyelin-509 (LysoSM-509) was measured in control subjects and plasma samples of predominantly untreated patients affected with lipid storage disorders (n=74). In addition, the oxysterols cholestane-3β,5α,6β-triol and 7-ketocholesterol were measured in a subset of these patients (n=36) as well as chitotriosidase activity (n=43). A systematic review of the literature was performed to assess the usefulness of these biochemical markers. Specific elevations of metabolites, i.e. without overlap between controls and other lipid storage disorders, were found for several lysosomal storage diseases: increased LysoSM levels in acid sphingomyelinase deficiency (Niemann-Pick disease type A/B), LysoGb3 levels in males with classical phenotype Fabry disease and LysoHexCer (i.e. lysoglucosylceramide/lysogalactosylceramide) in Gaucher and Krabbe diseases. While elevated levels of LysoSM-509 and cholestane-3β,5α,6β-triol did not discriminate between Niemann Pick disease type C and acid sphingomyelinase deficiency, LysoSM-509/LysoSM ratio was specifically elevated in Niemann-Pick disease type C. In Gaucher disease type I, mild increases in several lysosphingolipids were found including LysoGb3 with levels in the range of non-classical Fabry males and females. Chitotriosidase showed specific elevations in symptomatic Gaucher disease, and was mildly

Iron storage in liver, bone marrow and splenic Gaucheroma reflects residual disease in type 1 Gaucher disease patients on treatment.

Science.gov (United States)

Regenboog, Martine; Bohte, Anneloes E; Akkerman, Erik M; Stoker, Jaap; Hollak, Carla E M

2017-11-01

Gaucher disease (GD) is a lysosomal storage disorder characterized by the storage of glycosphingolipids in macrophages. Despite effective therapy, residual disease is present in varying degrees and may be associated with late complications, such as persistent bone or liver disease and increased cancer risk. Gaucher macrophages are capable of storing iron and locations of residual disease may thus be detectable with iron imaging. Forty type 1 GD (GD1) patients and 40 matched healthy controls were examined using a whole-body magnetic resonance imaging protocol consisting of standard sequences, allowing analysis of iron content per organ, expressed as R2* (Hz). Median R2* values were significantly elevated in GD1 patients as compared to healthy controls in liver [41 Hz (range 29-165) vs. 38 Hz (range 28-53), P Gaucher lesions known as Gaucheroma were found to have increased R2* values. R2* values of liver, spleen and vertebral bone marrow strongly correlated with serum ferritin levels. GD1 patients with persistent hyperferritinaemia demonstrate increased iron levels in liver and bone marrow, which may carry a risk for liver fibrosis and cancer. © 2017 John Wiley & Sons Ltd.

Diffuse reticuloendothelial system involvement in type IV glycogen storage disease with a novel GBE1 mutation: a case report and review.

Science.gov (United States)

Magoulas, Pilar L; El-Hattab, Ayman W; Roy, Angshumoy; Bali, Deeksha S; Finegold, Milton J; Craigen, William J

2012-06-01

Glycogen storage disease type IV is a rare autosomal recessive disorder of glycogen metabolism caused by mutations in the GBE1 gene that encodes the 1,4-alpha-glucan-branching enzyme 1. Its clinical presentation is variable, with the most common form presenting in early childhood with primary hepatic involvement. Histologic manifestations in glycogen storage disease type IV typically consist of intracytoplasmic non-membrane-bound inclusions containing abnormally branched glycogen (polyglucosan bodies) within hepatocytes and myocytes. We report a female infant with classic hepatic form of glycogen storage disease type IV who demonstrated diffuse reticuloendothelial system involvement with the spleen, bone marrow, and lymph nodes infiltrated by foamy histiocytes with intracytoplasmic polyglucosan deposits. Sequence analysis of the GBE1 gene revealed compound heterozygosity for a previously described frameshift mutation (c.1239delT) and a novel missense mutation (c.1279G>A) that is predicted to alter a conserved glycine residue. GBE enzyme analysis revealed no detectable activity. A review of the literature for glycogen storage disease type IV patients with characterized molecular defects and deficient enzyme activity reveals most GBE1 mutations to be missense mutations clustering in the catalytic enzyme domain. Individuals with the classic hepatic form of glycogen storage disease type IV tend to be compound heterozygotes for null and missense mutations. Although the extensive reticuloendothelial system involvement that was observed in our patient is not typical of glycogen storage disease type IV, it may be associated with severe enzymatic deficiency and a poor outcome. Copyright © 2012 Elsevier Inc. All rights reserved.

Review: Cytokines in Gaucher disease: Role in the pathogenesis of ...

African Journals Online (AJOL)

Gaucher disease (GD) is the most frequently encountered lysosomal storage disease caused by inborn defects of themembrane-bound lysosomal enzyme, acid b-glucosidase or glucocerebrosidase. This defective activity causes an accumulation of glucocerebroside (glucosylceramide) in the lysosomes of cells derived from ...

Magnetic resonance findings of the corpus callosum in canine and feline lysosomal storage diseases.

Science.gov (United States)

Hasegawa, Daisuke; Tamura, Shinji; Nakamoto, Yuya; Matsuki, Naoaki; Takahashi, Kimimasa; Fujita, Michio; Uchida, Kazuyuki; Yamato, Osamu

2013-01-01

Several reports have described magnetic resonance (MR) findings in canine and feline lysosomal storage diseases such as gangliosidoses and neuronal ceroid lipofuscinosis. Although most of those studies described the signal intensities of white matter in the cerebrum, findings of the corpus callosum were not described in detail. A retrospective study was conducted on MR findings of the corpus callosum as well as the rostral commissure and the fornix in 18 cases of canine and feline lysosomal storage diseases. This included 6 Shiba Inu dogs and 2 domestic shorthair cats with GM1 gangliosidosis; 2 domestic shorthair cats, 2 familial toy poodles, and a golden retriever with GM2 gangliosidosis; and 2 border collies and 3 chihuahuas with neuronal ceroid lipofuscinoses, to determine whether changes of the corpus callosum is an imaging indicator of those diseases. The corpus callosum and the rostral commissure were difficult to recognize in all cases of juvenile-onset gangliosidoses (GM1 gangliosidosis in Shiba Inu dogs and domestic shorthair cats and GM2 gangliosidosis in domestic shorthair cats) and GM2 gangliosidosis in toy poodles with late juvenile-onset. In contrast, the corpus callosum and the rostral commissure were confirmed in cases of GM2 gangliosidosis in a golden retriever and canine neuronal ceroid lipofuscinoses with late juvenile- to early adult-onset, but were extremely thin. Abnormal findings of the corpus callosum on midline sagittal images may be a useful imaging indicator for suspecting lysosomal storage diseases, especially hypoplasia (underdevelopment) of the corpus callosum in juvenile-onset gangliosidoses.

Magnetic resonance findings of the corpus callosum in canine and feline lysosomal storage diseases.

Directory of Open Access Journals (Sweden)

Daisuke Hasegawa

Full Text Available Several reports have described magnetic resonance (MR findings in canine and feline lysosomal storage diseases such as gangliosidoses and neuronal ceroid lipofuscinosis. Although most of those studies described the signal intensities of white matter in the cerebrum, findings of the corpus callosum were not described in detail. A retrospective study was conducted on MR findings of the corpus callosum as well as the rostral commissure and the fornix in 18 cases of canine and feline lysosomal storage diseases. This included 6 Shiba Inu dogs and 2 domestic shorthair cats with GM1 gangliosidosis; 2 domestic shorthair cats, 2 familial toy poodles, and a golden retriever with GM2 gangliosidosis; and 2 border collies and 3 chihuahuas with neuronal ceroid lipofuscinoses, to determine whether changes of the corpus callosum is an imaging indicator of those diseases. The corpus callosum and the rostral commissure were difficult to recognize in all cases of juvenile-onset gangliosidoses (GM1 gangliosidosis in Shiba Inu dogs and domestic shorthair cats and GM2 gangliosidosis in domestic shorthair cats and GM2 gangliosidosis in toy poodles with late juvenile-onset. In contrast, the corpus callosum and the rostral commissure were confirmed in cases of GM2 gangliosidosis in a golden retriever and canine neuronal ceroid lipofuscinoses with late juvenile- to early adult-onset, but were extremely thin. Abnormal findings of the corpus callosum on midline sagittal images may be a useful imaging indicator for suspecting lysosomal storage diseases, especially hypoplasia (underdevelopment of the corpus callosum in juvenile-onset gangliosidoses.

Magnetic Resonance Findings of the Corpus Callosum in Canine and Feline Lysosomal Storage Diseases

Science.gov (United States)

Hasegawa, Daisuke; Tamura, Shinji; Nakamoto, Yuya; Matsuki, Naoaki; Takahashi, Kimimasa; Fujita, Michio; Uchida, Kazuyuki; Yamato, Osamu

2013-01-01

Several reports have described magnetic resonance (MR) findings in canine and feline lysosomal storage diseases such as gangliosidoses and neuronal ceroid lipofuscinosis. Although most of those studies described the signal intensities of white matter in the cerebrum, findings of the corpus callosum were not described in detail. A retrospective study was conducted on MR findings of the corpus callosum as well as the rostral commissure and the fornix in 18 cases of canine and feline lysosomal storage diseases. This included 6 Shiba Inu dogs and 2 domestic shorthair cats with GM1 gangliosidosis; 2 domestic shorthair cats, 2 familial toy poodles, and a golden retriever with GM2 gangliosidosis; and 2 border collies and 3 chihuahuas with neuronal ceroid lipofuscinoses, to determine whether changes of the corpus callosum is an imaging indicator of those diseases. The corpus callosum and the rostral commissure were difficult to recognize in all cases of juvenile-onset gangliosidoses (GM1 gangliosidosis in Shiba Inu dogs and domestic shorthair cats and GM2 gangliosidosis in domestic shorthair cats) and GM2 gangliosidosis in toy poodles with late juvenile-onset. In contrast, the corpus callosum and the rostral commissure were confirmed in cases of GM2 gangliosidosis in a golden retriever and canine neuronal ceroid lipofuscinoses with late juvenile- to early adult-onset, but were extremely thin. Abnormal findings of the corpus callosum on midline sagittal images may be a useful imaging indicator for suspecting lysosomal storage diseases, especially hypoplasia (underdevelopment) of the corpus callosum in juvenile-onset gangliosidoses. PMID:24386203

Changes of sour taste and the composition of carboxylic acids induced in brewed coffee by γ-irradiation on green beans and storage of roast beans

International Nuclear Information System (INIS)

Tomoda, Goro; Matsuyama, Jun; Nagano, Akiko; Namatame, Mitsuko; Morita, Yoshiaki.

1980-01-01

Brazil santos green coffee beans were irradiated with 60 Co-γ rays at doses of 0, 0.05, 0.5 and 1.5 Mrad respectively and changes of the composition of carboxylic acids in roast beans were analyzed by means of GLC together with those of the organoleptic properties of roast beans during storage by use of the cup testing. The total acid content immediately after roasting was about 6,000 mg/100 g (roast beans) and the composition of carboxylic acids was as follows. Chlorogenic acid: hydroxy-carboxylic acids: mono-carboxylic acid: others = 73 : 18 : 7 : 2. Fresh coffee flavour was influenced markedly especially in acid taste by both irradiation of γ-rays on green beans and storage of roast beans, because of the change of above acids composition. On γ-ray irradiation, the change of the acid composition were more clear than that of stored roast beans. Therefore, the quality of γ-irradiated coffee beans seems to be closely associated with the ratio of hydroxy-carboxylic acids mg/ monocarboxylic acids mg, but little with total acid content. (author)

Laparoscopic surgery for gastro-esophageal acid reflux disease

NARCIS (Netherlands)

Schijven, Marlies P.; Gisbertz, Suzanne S.; van Berge Henegouwen, Mark I.

2014-01-01

Gastro-esophageal reflux disease is a troublesome disease for many patients, severely affecting their quality of life. Choice of treatment depends on a combination of patient characteristics and preferences, esophageal motility and damage of reflux, symptom severity and symptom correlation to acid

Effect of home processing and storage on ascorbic acid and beta-carotene content of Bathua (Chenopodium album) and fenugreek (Trigonella foenum graecum) leaves.

Science.gov (United States)

Yadav, S K; Sehgal, S

1997-01-01

The present investigation was conducted to study the effect of selected processing and storage methods on the concentration of ascorbic acid and beta-carotene in Bathua and fenugreek leaves. Methods included storage of leaves with or without polythene bags for 24 and 48 h in a refrigerator at 5 degrees C; at 30 degrees C in polythene bags; drying (sun and oven); blanching (5, 10, 15 min); open pan and pressure cooking. Ascorbic acid content of fresh leaves was 220.97 to 377.65 mg and beta-carotene content was 19.00 to 24.64 mg/100 g, DW. The percent loss of ascorbic acid ranged from 2.03 to 8.77 and 45.15 to 66.9 while lower losses (0.0 to 1.75 and 1.63 to 2.84) of beta-carotene were observed in leaves stored in the refrigerator and at 30 degrees C, respectively. A markedly greater reduction in ascorbic acid and beta-carotene was observed in dried, blanched and cooked leaves. The study data suggest that storage of leaves in refrigeration, drying in oven, blanching for a short time and cooking in a pressure cooker results in better retention of these two vitamins.

[Differentiation therapy for non-acidic gastroesophageal reflux disease].

Science.gov (United States)

Lishchuk, N B; Simanenkov, V I; Tikhonov, S V

2017-01-01

To investigate the clinical and pathogenetic features of the non-acidic types of gastroesophageal reflux disease (GERD) and to evaluate the impact of combined therapy versus monotherapy on the course of this disease. The investigation enrolled 62 patients with non-acidic GERD. The follow-up period was 6 weeks. The patients were divided into 2 groups: 1) weakly acidic gastroesophageal refluxes (GER); 2) weakly alkaline GER. Then each group was distributed, thus making up 4 groups: 1) 19 patients with weakly acidic GER who received monotherapy with rabeprazole 20 mg/day; 2) 21 patients with weakly acidic GER had combined therapy with rabeprazole 20 mg and itopride; 3) 8 patients with weakly alkaline GER who received ursodeoxycholic acid (UDCA) monotherapy; and 4) 14 patients with weakly alkaline GER who had combined therapy with UDCA and itopride, The clinical symptoms of the disease, the endoscopic pattern of the upper gastrointestinal tract (GIT) mucosa, histological changes in the esophageal and gastric mucosa, and the results of 24-hour impedance pH monitoring were assessed over time. During differentiation therapy, the majority of patients reported positive clinical changes and an improved or unchanged endoscopic pattern. Assessment of impedance pH monitoring results revealed decreases in the overall number of GERs, the presence of a bolus in the esophagus, and the number of proximal refluxes. These changes were noted not only in patients taking proton pump inhibitors (PPIs), but also in those treated with UDCA monotherapy or combined PPI and prokinetic therapy. A differentiated approach to non-acidic GER treatment contributes to its efficiency. Adding the prokinetic itomed (itopride hydrochloride) to PPI therapy in a patient with weakly acidic GER enhances the efficiency of treatment, by positively affecting upper GIT motility. The mainstay of therapy for GERD with a predominance of weakly alkaline refluxes is UDCA, the combination of the latter and the

Regional oesophageal sensitivity to acid and weakly acidic reflux in patients with non-erosive reflux disease.

Science.gov (United States)

Emerenziani, S; Ribolsi, M; Sifrim, D; Blondeau, K; Cicala, M

2009-03-01

The mechanisms underlying symptoms in non-erosive reflux disease (NERD) remain to be elucidated. Non-erosive reflux disease patients appear to be more sensitive to intraluminal stimula than erosive patients, the proximal oesophagus being the most sensitive. In order to assess regional oesophageal changes in reflux acidity and sensitivity to reflux, according either to the acidity or the composition of the refluxate, combined multiple pH and multiple pH-impedance (pH-MII) was performed in 16 NERD patients. According to multiple pH-metry, 29% and 12% of reflux events reached the middle and proximal oesophagus respectively, and 35% and 19% according to conventional pH-MII (P acid reflux became weakly acidic at the proximal oesophagus. In all patients, the frequency of symptomatic refluxes, both acid and weakly acidic, was significantly higher at the proximal, compared with distal oesophagus (25 +/- 8%vs 11 +/- 2% for acid reflux and 27 +/- 8%vs 8 +/- 2% for weakly acidic reflux; P reflux. As approximately 30% of acid reflux becomes weakly acidic along the oesophageal body, to better characterize proximal reflux, in clinical practice, combined proximal pH-impedance monitoring should be used. In NERD patients, the proximal oesophagus seems to be more sensitive to both acid and weakly acidic reflux.

Bone marrow transplantation in patients with storage diseases: a developing country experience

Directory of Open Access Journals (Sweden)

Lange Marcos C.

2006-01-01

Full Text Available Bone marrow transplantation (BMT is a therapeutic option for patients with genetic storage diseases. Between 1979 and 2002, eight patients, four females and four males (1 to 13 years old were submitted to this procedure in our center. Six patients had mucopolysaccharidosis (MPS I in 3; MPS III in one and MPS VI in 2, one had adrenoleukodystrophy (ALD and one had Gaucher disease. Five patients had related and three unrelated BMT donor. Three patients developed graft versus host disease (two MPS I and one MPS VI and died between 37 and 151 days after transplantation. Five patients survived 4 to 16 years after transplantation. Three patients improved (one MPS I; one MPS VI and the Gaucher disease patient, one patient had no disease progression (ALD and in one patient this procedure did not change the natural course of the disease (MPS III.

Enhancing Lipid Stability in Irradiated Beef Mince by Oleoresins and/ or Ascorbic Acid during Chilling Storage

International Nuclear Information System (INIS)

Zahran, D.A.

2008-01-01

Lipid Oxidation, fatty acids profile and sensory properties of irradiated beef mince (2.5 kGy) treated with oleoresins (rosemary or ginger), ascorbic acid, or combination of ascorbic acid and oleoresins were investigated during 30 days of chilled storage. Thiobarbituric acid reactive substances (TBARS) as an indication of lipid oxidation, of irradiated control samples were significantly higher than those of non irradiated control and samples treated with rosemary and ginger oleoresins. By GC-MS analysis, it was found that the relative percentage of total saturated fatty acids (TSFA) increased in all treatments. However, the highest increase was recorded in irradiated control samples compared to non irradiated control samples. Beef mince samples treated with oleoresins (rosemary or ginger) had the best scores for discoloration and off odour. Thus, the addition of oleoresins (rosemary or ginger) to beef mince before irradiation could be an easily applied method to minimize oxidative degradation of irradiated meat

Osteochondritis dissecans (OCD), an endoplasmic reticulum storage disease?

DEFF Research Database (Denmark)

Skagen, Peter Storgaard; Horn, T; Kruse, H A

2011-01-01

Osteochondritis dissecans (OCD) fragments, cartilage and blood from four patients were used for morphological and molecular analysis. Controls included articular cartilage and blood samples from healthy individuals. Light microscopy and transmission electron microscopy (TEM) showed abnormalities...... in chondrocytes and extracellular matrix of cartilage from OCD patients. Abnormal type II collagen heterofibrils in "bundles" and chondrocytes with abnormal accumulation of matrix proteins in distended rough endoplasmic reticulum were typical findings. Further, Von Kossa staining and TEM showed empty lacunae...... polymorphism was found within the COL2A1 gene for one patient. We suggest that OCD lesions are caused by an alteration in chondrocyte matrix synthesis causing an endoplasmic reticulum storage disease phenotype, which disturbs or abrupts endochondral ossification....

Thermal properties and heat storage analysis of palmitic acid-TiO_2 composite as nano-enhanced organic phase change material (NEOPCM)

International Nuclear Information System (INIS)

Sharma, R.K.; Ganesan, P.; Tyagi, V.V.; Metselaar, H.S.C.; Sandaran, S.C.

2016-01-01

Highlights: • Novel composite of palmitic acid and TiO_2 nanoparticles with enhanced thermal energy storage capabilities • The composite is thermally reliable and chemically stable. • Thermal conductivity of the composite increases significantly with the loading. - Graphical Abstract: - Abstract: In the present study, the phase change behavior of prepared novel composites of palmitic acid and solid nanoparticles of titanium dioxide (TiO_2) for thermal energy storage has been investigated. The nanoparticles are dispersed into the base fluid in various mass fractions (0.5, 1, 3, and 5%), and their effects on the thermo-physical properties have been investigated. Structural analysis has been carried out by using FESEM, and crystallography was checked by XRD technique. The chemical/functional groups of the base fluid and composite PCMs have been analyzed by using FT-IR spectrum. The observations showed that the TiO_2 nanoparticles do not affect the chemical structure of palmitic acid; however they improve the chemical stability. The phase transition temperature and latent heat of fusion has shown the significant stability with the increase in nanoparticle weight fractions. The accelerated thermal cycle test of the composite shows good thermal reliability for 1500 melt/freeze cycles. Thermal conductivity of palmitic acid increased gradually by 12.7, 20.6, 46.6, and 80% for the nanoparticle weight fractions of 0.5, 1, 3, and 5% respectively. Based on the results, it can be mentioned that the prepared palmitic acid based nano-enhanced organic phase change composite materials can be very well used as potential solar thermal energy storage materials.

Use of FTA cards for the storage of breast carcinoma nucleic acid on fine-needle aspiration samples.

Science.gov (United States)

Peluso, Anna Lucia; Cascone, Anna Maria; Lucchese, Lucrezia; Cozzolino, Immacolata; Ieni, Antonio; Mignogna, Chiara; Pepe, Stefano; Zeppa, Pio

2015-10-01

The preservation and storage of nucleic acids is important for DNA molecular techniques. The material obtained by fine-needle aspiration (FNA) is often scanty and can not be wasted. FTA cards are filter papers that immobilize and stabilize nucleic acids and can be stored at room temperature. The current study evaluated whether nucleic acids of breast carcinoma cells, obtained by FNA in a clinical setting, may be collected, stored, and preserved on FTA cards. Thirty breast carcinoma, 5 non-Hodgkin lymphoma (NHL), and 5 benign reactive lymph node (RLN) cell samples obtained by FNA were stored at -80 °C and on FTA cards. DNA extraction and polymerase chain reaction were performed on cells at -80 °C and on 2 punched disks of FTA cards. Fifty nanograms of extracted DNA from both sample types were used to amplify the Janus Kinase 2 (JAK2) gene. The mean value of DNA extracted from breast carcinoma cells was 28.19 ng/µL for that stored at -80 °C and 3.28 ng/µL for that stored on FTA cards. Agarose gel analysis demonstrated expected bands of DNA in 29 cases (97%) with both methods. The mean value of DNA extracted from NHL and RLN samples was 37.54 ng/µL and 4.28 ng/µL, respectively, and agarose gel analysis demonstrated bands of high molecular weight DNA in both methods. Significant differences in DNA yield were found between storage at -80 °C and FTA cards (PFTA cards can be conveniently used for the storage of breast carcinoma cells obtained by FNA, thus providing a reliable alternative to traditional methods. © 2015 American Cancer Society.

Yield and acidity indices of sunflower and soybean oils in function of grain drying and storage

Directory of Open Access Journals (Sweden)

Paulo Carteri Coradi

2017-04-01

Full Text Available The aim of this study was to identify the best conditions for drying and storing soybeans and sunflower grains to maintain their quality. In the first experiment, the soybeans were found to have initial moisture contents of 25 and 19% (w.b. at different drying air temperatures (75, 90, 105, and 120°C. In the second step, the soybeans were evaluated after they were stored in paper bags and plastic polyethylene at temperatures of 3, 10 and 23°C for six months. In the third experiment, sunflower grains were tested after exposure to drying air temperatures of 45, 55, 65, and 75°C, and under storage conditions of 25°C and 50%, 20°C and 60%, 30°C and 40% RH over six months in paper bags and raffia. Drying the sunflower seeds at 45°C and storing them at 30°C and 40% RH led to higher oil yields and lower acid numbers. The oil that was extracted from the acid number was higher for soybean grains that were dried down from initial concentrations of 25% water at a drying air temperature of 120°C. The air temperature in storage at 3°C favored for yield and reduction of the soybean oil acidity.

Effect of gamma radiation treatment on some fungi causing storage diseases of banana fruits

International Nuclear Information System (INIS)

EL-Ashmawi, A.M.M.

1982-01-01

Banana is one of the most popular fruits in many tropical and sub-tropical countries. in recent years, the quality of egyptian banana markedly declined. A major factor contributing to this decline is the development of fruit rot, which is the most widely occurring disease either in the field or in storage. Different fungi attack banana fruits causing considerable losses. Most of the fungi responsible for post harvest rots of banana are usually carried from the field, on the surface of the fruit itself or in injured and rotting fruits causing severe rats during storage. These rots make the fruits difficult to handle and undesirable to the consumers. Botryodiplodia theobromae is known to be the most important pathogen responsible for the infection in storage

Farber's Disease

Science.gov (United States)

... management, and therapy of rare diseases, including the lipid storage diseases. Research on lipid storage diseases within the Network includes ... management, and therapy of rare diseases, including the lipid storage diseases. Research on lipid storage diseases within the Network includes ...

Glycogen Storage Disease Type Ia in Canines: A Model for Human Metabolic and Genetic Liver Disease

OpenAIRE

Specht, Andrew; Fiske, Laurie; Erger, Kirsten; Cossette, Travis; Verstegen, John; Campbell-Thompson, Martha; Struck, Maggie B.; Lee, Young Mok; Chou, Janice Y.; Byrne, Barry J.; Correia, Catherine E.; Mah, Cathryn S.; Weinstein, David A.; Conlon, Thomas J.

2011-01-01

A canine model of Glycogen storage disease type Ia (GSDIa) is described. Affected dogs are homozygous for a previously described M121I mutation resulting in a deficiency of glucose-6-phosphatase-α. Metabolic, clinicopathologic, pathologic, and clinical manifestations of GSDIa observed in this model are described and compared to those observed in humans. The canine model shows more complete recapitulation of the clinical manifestations seen in humans including “lactic acidosis”, larger size,...

Omega-3 polyunsaturated fatty acids for cardiovascular diseases: present, past and future.

Science.gov (United States)

Watanabe, Yasuhiro; Tatsuno, Ichiro

2017-08-01

Large-scale epidemiological studies on Greenlandic, Canadian and Alaskan Eskimos have examined the health benefits of omega-3 fatty acids consumed as part of the diet, and found statistically significant relative reduction in cardiovascular risk in people consuming omega-3 fatty acids. Areas covered: This article reviews studies on omega-3 fatty acids during the last 50 years, and identifies issues relevant to future studies on cardiovascular (CV) risk. Expert commentary: Although a meta-analysis of large-scale prospective cohort studies and randomized studies reported that fish and fish oil consumption reduced coronary heart disease-related mortality and sudden cardiac death, omega-3 fatty acids have not yet been shown to be effective in secondary prevention trials on patients with multiple cardiovascular disease (CVD) risk factors. The ongoing long-term CV interventional outcome studies investigate high-dose, prescription-strength omega-3 fatty acids. The results are expected to clarify the potential role of omega-3 fatty acids in reducing CV risk. The anti-inflammatory properties of omega-3 fatty acids are also important. Future clinical trials should also focus on the role of these anti-inflammatory mediators in human arteriosclerotic diseases as well as inflammatory diseases.

Chemical heat pump and chemical energy storage system

Science.gov (United States)

Clark, Edward C.; Huxtable, Douglas D.

1985-08-06

A chemical heat pump and storage system employs sulfuric acid and water. In one form, the system includes a generator and condenser, an evaporator and absorber, aqueous acid solution storage and water storage. During a charging cycle, heat is provided to the generator from a heat source to concentrate the acid solution while heat is removed from the condenser to condense the water vapor produced in the generator. Water is then stored in the storage tank. Heat is thus stored in the form of chemical energy in the concentrated acid. The heat removed from the water vapor can be supplied to a heat load of proper temperature or can be rejected. During a discharge cycle, water in the evaporator is supplied with heat to generate water vapor, which is transmitted to the absorber where it is condensed and absorbed into the concentrated acid. Both heats of dilution and condensation of water are removed from the thus diluted acid. During the discharge cycle the system functions as a heat pump in which heat is added to the system at a low temperature and removed from the system at a high temperature. The diluted acid is stored in an acid storage tank or is routed directly to the generator for reconcentration. The generator, condenser, evaporator, and absorber all are operated under pressure conditions specified by the desired temperature levels for a given application. The storage tanks, however, can be maintained at or near ambient pressure conditions. In another form, the heat pump system is employed to provide usable heat from waste process heat by upgrading the temperature of the waste heat.

On the Effectiveness of Pantoprazole Use in Acid-Related Diseases in Children

Directory of Open Access Journals (Sweden)

O.H. Shadrin

2016-04-01

Full Text Available The article considers the place of pantoprazole in acid-related diseases in children. It is emphasized that in practical gastroenterology, in addition to the original proton pump inhibitors (brands, generic drugs are also used, which are more available for most patients, unlike the original proton pump inhibitors. The objective of the work presented in the article was to study the clinical efficacy of the generic drug pantoprazole (Zolopant in the treatment of acid-related diseases in children. Zolopant proved to be a highly effective antisecretory drug for the treatment of acid-related diseases (chronic gastroduodenitis, peptic ulcer disease, gastroesophageal reflex disease in children, which is well tolerated by children and does not cause adverse reactions.

Eutectic mixtures of some fatty acids for latent heat storage: Thermal properties and thermal reliability with respect to thermal cycling

International Nuclear Information System (INIS)

Sari, Ahmet

2006-01-01

Accelerated thermal cycle tests have been conducted to study the change in melting temperatures and latent heats of fusion of the eutectic mixtures of lauric acid (LA)-myristic acid (MA), lauric acid (LA)-palmitic acid (PA) and myristic acid (MA)-stearic acid (SA) as latent heat storage materials. The thermal properties of these materials were determined by the differential scanning calorimetry (DSC) analysis method. The thermal reliability of the eutectic mixtures after melt/freeze cycles of 720, 1080 and 1460 was also evaluated using the DSC curves. The accelerated thermal cycle tests indicate that the melting temperatures usually tend to decrease, and the variations in the latent heats of fusion are irregular with increasing number of thermal cycles. Moreover, the probable reasons for the change in thermal properties of the eutectic mixtures after repeated thermal cycles were investigated. Fourier Transform Infrared (FT-IR) spectroscopic analysis indicates that the accelerated melt/freeze processes do not cause any degradation in the chemical structure of the mixtures. The change in thermal properties of the eutectic mixtures with increasing number of thermal cycles is only because of the presence of certain amounts of impurities in the fatty acids used in their preparation. It is concluded that the tested eutectic mixtures have reasonable thermal properties and thermal reliability as phase change materials (PCMs) for latent heat storage in any solar heating applications that include a four year utilization period

Dietary fatty acids linking postprandial metabolic response and chronic diseases.

Science.gov (United States)

Ortega, Almudena; Varela, Lourdes M; Bermudez, Beatriz; Lopez, Sergio; Abia, Rocio; Muriana, Francisco J G

2012-01-01

Chronic diseases are by far one of the main causes of mortality in the world. One of the current global recommendations to counteract disability and premature death resulting from chronic diseases is to decrease the consumption of energy-dense high-fat diets, particularly those rich in saturated fatty acids (SFA). The most effective replacement for SFA in terms of risk factor outcomes for chronic disease are polyunsaturated fatty acids (PUFA) and monounsaturated fatty acids (MUFA). The biochemical basis for healthy benefits of such a dietary pattern has been widely evaluated under fasting conditions. However, the increasing amount of data available from multiple studies suggest that the postprandial state, i.e., "the period that comprises and follows a meal", plays an important, yet underappreciated, role in the genesis of numerous pathological conditions. In this review, the potential of MUFA, PUFA, and SFA to postprandially affect selected metabolic abnormalities related to chronic diseases is discussed.

Effect of storage on the amino acid composition and biological quality of irradiated macacar beans Vigna unguiculata (L.) Walp

International Nuclear Information System (INIS)

Coelho, L.C.B.B.; de Medeiros, R.B.; Flores, H.

1978-01-01

The effects of two doses of gamma radiation (100 and 1,000 krad) upon the stability over a 6-month storage period of the amino acid composition and protein efficiency ratio (PER) of the macacar bean Vigna unguiculata (L.) Walp were investigated. No important differences were noted when the aminograms of irradiated and nonirradiated beans, either raw or cooked were compared. Nevertheless, the losses of lysine, arginine, and histidine due to cooking were greater in the irradiated beans. The PER of nonirradiated was higher than that of irradiated beans before and after the 6 months of storage, and was always lowest in the beans subjected to the higher dose of radiation. Qualitatively, an association was observed between the nutritional value (PER) and small decreases in the content of certain amino acids which resulted mainly from increased thermal lability of the irradiated bean protein

Palmitic acid/polypyrrole composites as form-stable phase change materials for thermal energy storage

International Nuclear Information System (INIS)

Silakhori, Mahyar; Metselaar, Hendrik Simon Cornelis; Mahlia, Teuku Meurah Indra; Fauzi, Hadi; Baradaran, Saeid; Naghavi, Mohammad Sajad

2014-01-01

Highlights: • A novel phase change composite of palmitic acid–polypyrrole(PA–PPy) was fabricated. • Thermal properties of PA–PPy are characterized in different mass ratios of PA–PPy. • Thermal cycling test showed that form stable PCM had a favorable thermal reliability. - Abstract: In this study a novel palmitic acid (PA)/polypyrrole (PPy) form-stable PCMs were readily prepared by in situ polymerization method. PA was used as thermal energy storage material and PPy was operated as supporting material. Form-stable PCMs were investigated by SEM (scanning electron microscopy) and FTIR (Fourier transform infrared spectrometer) analysis that illustrated PA Particles were wrapped by PPy particles. XRD (X-ray diffractometer) was used for crystalline phase of PA/PPy composites. Thermogravimetry analysis (TGA) and differential scanning calorimetry (DSC) were used for investigating Thermal stability and thermal energy storage properties of prepared form-stable PCMs. According to the obtained results the form stable PCMs exhibited favorable thermal stability in terms of their phase change temperature. The form-stable PCMs (79.9 wt% loading of PA) were considered as the highest loading PCM with desirable latent heat storage of 166.3 J/g and good thermal stability. Accelerated thermal cycling tests also showed that form stable PCM had an acceptable thermal reliability. As a consequence of acceptable thermal properties, thermal stability and chemical stability, we can consider the new kind of form stable PCMs for low temperature solar thermal energy storage applications

OMEGA-3 FATTY ACIDS AND AGE-RELATED DISEASES: REALITIES AND PROSPECTS

Directory of Open Access Journals (Sweden)

O. M. Drapkina

2015-01-01

Full Text Available Efficacy of omega-3 fatty acids in cardiology is so high that in many countries omega-3 fatty acids are included into the treatment protocols for patients with cardiovascular diseases. This therapeutic class slows down oxidative stress and chronic inflammation processes, thereby providing a significant contribution to the complex treatment of hypertension. Besides, omega-3 fatty acids slow down the aging process and prevent the development of age-related diseases affecting the rate of telomere shortening.

Systematic review: role of acid, weakly acidic and weakly alkaline reflux in gastro-oesophageal reflux disease

NARCIS (Netherlands)

Boeckxstaens, G. E.; Smout, A.

2010-01-01

The importance of weakly acidic and weakly alkaline reflux in gastro-oesophageal reflux disease (GERD) is gaining recognition. To quantify the proportions of reflux episodes that are acidic (pH <4), weakly acidic (pH 4-7) and weakly alkaline (pH >7) in adult patients with GERD, and to evaluate their

Bile acids in health and disease

DEFF Research Database (Denmark)

Krag, E; Thaysen, E H

1996-01-01

Over the last quarter of a century Danish research on bile acids has comprised studies of their physical and chemical properties, their physiology, pathophysiology, metabolism, and kinetics, and their clinical applicability. In the beginning of the period a major contribution was made to the unde......Over the last quarter of a century Danish research on bile acids has comprised studies of their physical and chemical properties, their physiology, pathophysiology, metabolism, and kinetics, and their clinical applicability. In the beginning of the period a major contribution was made...... to the understanding of the factors involved in the solubility of cholesterol in bile. The growing international understanding of the potential importance of the bile acids in health and disease gave raise to a substantial Danish contribution in the 1970s and 1980s in parallel with international achievements. Emphasis...... was on the possible clinical implications of bile acids. Studies on physiology and pathophysiology were in focus. Patients who have had an intestinal bypass operation for obesity served as a model for obtaining new knowledge on various aspects of the properties of the bile acids. Also the analytical methods were...

Serum uric acid and lipid profiles in sporadic Creutzfeldt-Jakob disease.

Science.gov (United States)

Chen, Shuai; He, Shuang; Shang, Jun-Kui; Ma, Ming-Ming; Xu, Chang-Shui; Shi, Xiao-Hong; Zhang, Jie-Wen

2016-02-01

Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive, and fatal neurodegenerative disease affecting the central nervous system. Brain lipid homeostasis and oxidative stress seem to play an important role in the disease pathogenesis. But little was known whether serum lipids and uric acid (a natural antioxidant) levels changed in patients with prion disease. Here we retrospectively reviewed and compared the serum lipids and uric acid levels of 19 probable sporadic CJD patients and 26 healthy control subjects. We found that the serum uric acid levels in sporadic CJD patients were significantly lower than that in controls (P=0.01). Serum triglycerides, cholesterol, low-density lipoprotein (LDL), high-density lipoprotein (HDL), and apolipoprotein A1 (ApoA1) were similar in sporadic CJD patients and controls. However, LDL/HDL ratio was lower in sporadic CJD patients (P=0.003). The low serum uric acid and LDL/HDL ratio levels in sporadic CJD indicate that dysfunction in the lipid homeostasis and oxidative stress is associated with sporadic prion disease. Copyright © 2015 The Canadian Society of Clinical Chemists. Published by Elsevier Inc. All rights reserved.

A simplified equivalent circuit model for simulation of Pb-acid batteries at load for energy storage application

Energy Technology Data Exchange (ETDEWEB)

Zhu, Wenhua H.; Zhu Ying [Center for Microfibrous Materials, Department of Chemical Engineering, 212 Ross Hall, Auburn University, AL 36849-5127 (United States); Tatarchuk, Bruce J., E-mail: brucet@eng.auburn.edu [Center for Microfibrous Materials, Department of Chemical Engineering, 212 Ross Hall, Auburn University, AL 36849-5127 (United States)

2011-08-15

Highlights: {yields} Pb-acid battery is reexamined in electrode structure and capacitance enhancement. {yields} Pb-acid batteries were tested through the electrochemical impedance at loads. {yields} Electrode behaviors are evaluated by simulation using an equivalent circuit model. {yields} A defective and a failed Pb-acid battery was used in non-destructive analysis. {yields} Potential applications are for power reserve and sustainable electricity storage. - Abstract: Three main types of battery chemistries in consideration for vehicle applications are Pb-acid, nickel-metal hydride, and lithium-ion batteries. Lead-acid batteries are widely used in traditional automotive applications for many years. Higher voltage, high-rate discharge capability, good specific energy, lower temperature performance, lower thermal management requirement, and low-cost in both manufacturing and recycling are the advantages of the rechargeable battery. Disadvantages of the lead-acid battery are: weight concerns of lead metal (lower energy density and lower power density) and limited cycle-life (especially in deep-cycle duties). If two major disadvantages have been significantly changed to a proper state to compete with other battery chemistries, the Pb-acid battery is still a good candidate in considering of cost/performance ratio. The lead-acid battery is always a good power source for fast starting of cold vehicles, for recharging from either a stop-start braking system, or for a charge from the engine itself, which consumes battery energy or stores electricity back into chemical energy. The main reasons for reexamining this battery chemistry are cost-savings and life-cycling considerations upon advances in electrode structure design and enhancement of capacitance behavior inside the battery pack. Several Pb-acid batteries were evaluated and tested through a unique method, i.e., the electrochemical impedance method at different loads, in order to characterize and further understand the

A simplified equivalent circuit model for simulation of Pb-acid batteries at load for energy storage application

International Nuclear Information System (INIS)

Zhu, Wenhua H.; Zhu Ying; Tatarchuk, Bruce J.

2011-01-01

Highlights: → Pb-acid battery is reexamined in electrode structure and capacitance enhancement. → Pb-acid batteries were tested through the electrochemical impedance at loads. → Electrode behaviors are evaluated by simulation using an equivalent circuit model. → A defective and a failed Pb-acid battery was used in non-destructive analysis. → Potential applications are for power reserve and sustainable electricity storage. - Abstract: Three main types of battery chemistries in consideration for vehicle applications are Pb-acid, nickel-metal hydride, and lithium-ion batteries. Lead-acid batteries are widely used in traditional automotive applications for many years. Higher voltage, high-rate discharge capability, good specific energy, lower temperature performance, lower thermal management requirement, and low-cost in both manufacturing and recycling are the advantages of the rechargeable battery. Disadvantages of the lead-acid battery are: weight concerns of lead metal (lower energy density and lower power density) and limited cycle-life (especially in deep-cycle duties). If two major disadvantages have been significantly changed to a proper state to compete with other battery chemistries, the Pb-acid battery is still a good candidate in considering of cost/performance ratio. The lead-acid battery is always a good power source for fast starting of cold vehicles, for recharging from either a stop-start braking system, or for a charge from the engine itself, which consumes battery energy or stores electricity back into chemical energy. The main reasons for reexamining this battery chemistry are cost-savings and life-cycling considerations upon advances in electrode structure design and enhancement of capacitance behavior inside the battery pack. Several Pb-acid batteries were evaluated and tested through a unique method, i.e., the electrochemical impedance method at different loads, in order to characterize and further understand the improved electrode

Enzyme replacement therapy in late-onset Pompe's disease : A three-year follow-up

NARCIS (Netherlands)

Winkel, LPF; Van den Hout, JMP; Kamphoven, JHJ; Disseldorp, JAM; Remmerswaal, M; Arts, WFM; Loonen, MCB; Vulto, AG; Van Doorn, PA; De Jong, G; Hop, W; Smit, GPA; Shapira, SK; Boer, MA; van Diggelen, OP; Reuser, AJJ; Van der Ploeg, AT

Pompe's disease is an autosomal recessive myopathy. The characteristic lysosomal storage of glycogen is caused by acid et-glucosidase deficiency. Patients with late-onset Pompe's disease present with progressive muscle weakness also affecting pulmonary function. In search of a treatment, we

Determination of Selected Amino Acids in Serum of Patients with Liver Disease.

Science.gov (United States)

Kanďár, Roman; Drábková, Petra; Toiflová, Tereza; Čegan, Alexander

2016-01-01

The determination of amino acids can be a reliable approach for extended diagnosis of liver diseases. This is because liver disease can be a cause of impaired amino acid metabolism. Therefore, a method for the determination of serum amino acids, applicable for clinical purposes, is necessary. The aim of this study was to find differences in the levels of selected amino acids between patients with liver disease and a control group. Samples of peripheral venous blood were obtained from a group of patients with liver disease (n = 131, 59 women at an average age of 60 years and 72 men at an average age of 52 years) and a control group (n = 105, 47 women at an average age of 62 years and 58 men at an average age of 58 years). Before the separation, the amino acids were derivatized with naphthalene-2,3-dicarboxaldehyde. For the separation, reverse phase column was used. The effluent was monitored with a fluorescence detector. There were significant differences in the concentrations of some amino acids between the patients and the control group, but also between women and men. Correlations between some amino acids and markers of liver blood tests and lipid metabolism were observed. A simple, relatively rapid and selective HPLC method with fluorescence detection for the determination of selected amino acids in serum has been developed.

Treatment of lysosomal storage disease in MPS VII mice using a recombinant adeno-associated virus.

Science.gov (United States)

Watson, G L; Sayles, J N; Chen, C; Elliger, S S; Elliger, C A; Raju, N R; Kurtzman, G J; Podsakoff, G M

1998-12-01

Mucopolysaccharidosis type VII (MPS VII) is a lysosomal storage disease caused by a genetic deficiency of beta-glucuronidase (GUS). We used a recombinant adeno-associated virus vector (AAV-GUS) to deliver GUS cDNA to MPS VII mice. The route of vector administration had a dramatic effect on the extent and distribution of GUS activity. Intramuscular injection of AAV-GUS resulted in high, localized production of GUS, while intravenous administration produced low GUS activity in several tissues. This latter treatment of MPS VII mice reduced glycosaminoglycan levels in the liver to normal and reduced storage granules dramatically. We show that a single administration of AAV-GUS can provide sustained expression of GUS in a variety of cell types and is sufficient to reverse the disease phenotype at least in the liver.

Preparation, morphology and thermal properties of electrospun fatty acid eutectics/polyethylene terephthalate form-stable phase change ultrafine composite fibers for thermal energy storage

International Nuclear Information System (INIS)

Cai Yibing; Ke Huizhen; Lin Liang; Fei Xiuzhu; Wei Qufu; Song Lei; Hu Yuan; Fong Hao

2012-01-01

Highlights: ► Electrospun binary fatty acid eutectics/PET ultrafine composite fibers were prepared. ► Fatty acid eutectics had appropriate phase transition temperature and heat enthalpy. ► Their morphological structures and thermal properties were different from each other. ► Composite fibers could be innovative form-stable PCMs for thermal energy storage. - Abstract: The ultrafine composite fibers based on the composites of binary fatty acid eutectics and polyethylene terephthalate (PET) with varied fatty acid eutectics/PET mass ratios (50/100, 70/100, 100/100 and 120/100) were fabricated using the technique of electrospinning as form-stable phase change materials (PCMs). The five binary fatty acid eutectics including LA–MA, LA–PA, MA–PA, MA–SA and PA–SA were prepared according to Schrader equation, and then were selected as an innovative type of solid–liquid PCMs. The results characterized by differential scanning calorimeter (DSC) indicated that the prepared binary fatty acid eutectics with low phase transition temperatures and high heat enthalpies for climatic requirements were more suitable for applications in building energy storage. The structural morphologies, thermal energy storage and thermal stability properties of the ultrafine composite fibers were investigated by scanning electron microscope (SEM), DSC and thermogravimetric analysis (TGA), respectively. SEM images revealed that the electrospun binary fatty acid eutectics/PET ultrafine composite fibers possessed the wrinkled surfaces morphologies compared with the neat PET fibers with cylindrical shape and smooth surfaces; the grooves or ridges on the corrugated surface of the ultrafine composite fibers became more and more prominent with increasing fatty acid eutectics amount in the composite fibers. The fibers with the low mass ratio maintained good structural morphologies while the quality became worse when the mass ratio is too high (more than 100/100). DSC measurements

Fabrication and characterization of stearic acid/polyaniline composite with electrical conductivity as phase change materials for thermal energy storage

International Nuclear Information System (INIS)

Wang, Yi; Ji, Hui; Shi, Huan; Zhang, Ting; Xia, TianDong

2015-01-01

Highlights: • Stearic acid/polyaniline composite PCM with electrical conductivity was fabricated. • Stearic acid acted as thermal energy storage media and doping acid. • Latent heats of SA/PANI are as high as the same type composites. • Improved electrical conductivity of capsules is 0.7042 S cm −1 . - Abstract: This paper presents the experimental investigation on the thermal properties and electrical conductivity of the new microencapsulated phase change material by entrapping of stearic acid (SA) into PANI (polyaniline) shell through self-assembly method. Experimental results reveal that PANI nuclei grew on the surface of SA, and then copied its original morphological structure and finally exhibited peony flower-like morphology. The two components have good compatibility and have no chemical reaction both in the process of fabrication and subsequent use, while hydrogen bondings between the imino groups and carboxyl groups exist. The maximum mass fraction of stearic acid loaded in SA/PANI is determined as high as 62.1 wt% without seepage of melted SA from capsules. Due to the secondary doping with carboxyl group, the composite phase change material embedded with SA exhibits improved electrical conductivity from 0.3968 S cm −1 to 0.7042 S cm −1 when compared to PANI. The phase change temperatures and latent heats of SA/PANI are measured to be 55.6 °C and 113.02 J/g for melting and, 50.8 °C and 112.58 J/g for freezing, respectively. TG analysis test revealed that the prepared SA/PANI composite PCM has high thermal durability in working temperature range. Moreover, the results of DSC, FT-IR, TG, conductivity investigation and thermal cycling test are all show that the thermal reliability and electrical conductivity of the SA/APNI have imperceptible changes. In total, the additional electrical conductivity, high heat storage potential and good thermal reliability and stability facilitated SA/PANI to be considered as a viable candidate for thermal

Ursodeoxycholic Acid in Treatment of Non-cholestatic Liver Diseases: A Systematic Review.

Science.gov (United States)

Reardon, Jillian; Hussaini, Trana; Alsahafi, Majid; Azalgara, Vladimir Marquez; Erb, Siegfried R; Partovi, Nilufar; Yoshida, Eric M

2016-09-28

Aims: To systematically evaluate the literature for evidence to support the use of bile acids in non-cholestatic liver conditions. Methods: Searches were conducted on the databases of Medline (1948-March 31, 2015), Embase (1980-March 31, 2015) and the Cochrane Central Register of Controlled Trials, and on Google and Google Scholar to identify articles describing ursodeoxycholic acid (UDCA) and its derivatives for non-cholestatic hepatic indications. Combinations of the following search terms were used: ursodeoxycholic acid, ursodiol, bile acids and/or salts, non alcoholic fatty liver, non alcoholic steatohepatitis, fatty liver, alcoholic hepatitis, alcohol, liver disease, autoimmune, autoimmune hepatitis, liver transplant, liver graft, transplant rejection, graft rejection, ischemic reperfusion injury, reperfusion injury, hepatitis B, hepatitis C, viral hepatitis, chronic hepatitis, acute hepatitis, transaminases, alanine transaminase, liver enzymes, aspartate aminotransferase, gamma-glutamyl transferase, gamma-glutamyl transpeptidase, bilirubin, alkaline phosphatase. No search limits were applied. Additionally, references of the included studies were reviewed to identify additional articles. Results: The literature search yielded articles meeting inclusion criteria for the following indications: non-alcoholic fatty liver disease (n = 5); alcoholic liver disease (n = 2); autoimmune hepatitis (n = 6), liver transplant (n = 2) and viral hepatitis (n = 9). Bile acid use was associated with improved normalization of liver biochemistry in non-alcoholic fatty liver disease, autoimmune hepatitis and hepatitis B and C infections. In contrast, liver biochemistry normalization was inconsistent in alcoholic liver disease and liver transplantation. The majority of studies reviewed showed that normalization of liver biochemistry did not correlate to improvement in histologic disease. In the prospective trials reviewed, adverse effects associated with the bile acids were limited

Lauric and palmitic acids eutectic mixture as latent heat storage material for low temperature heating applications

International Nuclear Information System (INIS)

Tuncbilek, Kadir; Sari, Ahmet; Tarhan, Sefa; Erguenes, Gazanfer; Kaygusuz, Kamil

2005-01-01

Palmitic acid (PA, 59.8 deg. C) and lauric acid (LA, 42.6 deg. C) are phase change materials (PCM) having quite high melting temperatures which can limit their use in low temperature solar applications such as solar space heating and greenhouse heating. However, their melting temperatures can be tailored to appropriate value by preparing a eutectic mixture of the lauric and the palmitic acids. In the present study, the thermal analysis based on differential scanning calorimetry (DSC) technique shows that the mixture of 69.0 wt% LA and 31 wt% PA forms a eutectic mixture having melting temperature of 35.2 deg. C and the latent heat of fusion of 166.3 J g -1 . This study also considers the experimental determination of the thermal characteristics of the eutectic mixture during the heat charging and discharging processes. Radial and axial temperature distribution, heat transfer coefficient between the heat transfer fluid (HTF) pipe and the PCM, heat recovery rate and heat charging and discharging fractions were experimentally established employing a vertical concentric pipe-in-pipe energy storage system. The changes of these characteristics were evaluated with respect to the effect of inlet HTF temperature and mass flow rate. The DSC thermal analysis and the experimental results indicate that the LA-PA eutectic mixture can be a potential material for low temperature thermal energy storage applications in terms of its thermo-physical and thermal characteristics

The Role of the Acid Pocket in Gastroesophageal Reflux Disease.

Science.gov (United States)

Mitchell, David R; Derakhshan, Mohammad H; Robertson, Elaine V; McColl, Kenneth E L

2016-02-01

Gastroesophageal reflux disease is one of the commonest chronic conditions in the western world and its prevalence is increasing worldwide. The discovery of the acid pocket explained the paradox of acid reflux occurring more frequently in the postprandial period despite intragastric acidity being low due to the buffering effect of the meal. The acid pocket was first described in 2001 when it was detected as an area of low pH immediately distal to the cardia using dual pH electrode pull-through studies 15 minutes after a meal. It was hypothesized that there was a local pocket of acid close to the gastroesophageal junction that escapes the buffering effect of the meal, and that this is the source of postprandial acidic reflux. The presence of the acid pocket has been confirmed in other studies using different techniques including high-resolution pHmetry, Bravo capsule, magnetic resonance imaging, and scintigraphy. This review aims to describe what we know about the acid pocket including its length, volume, fluid constituents, and its relationship to the lower esophageal sphincter and squamocolumnar junction. We will discuss the possible mechanisms that lead to the formation of the acid pocket and examine what differences exist in patients who suffer from acid reflux. Treatments for reflux disease that affect the acid pocket will also be discussed.

Amino Acid Catabolism in Alzheimer’s Disease Brain: Friend or Foe?

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Jeddidiah W. D. Griffin

2017-01-01

Full Text Available There is a dire need to discover new targets for Alzheimer’s disease (AD drug development. Decreased neuronal glucose metabolism that occurs in AD brain could play a central role in disease progression. Little is known about the compensatory neuronal changes that occur to attempt to maintain energy homeostasis. In this review using the PubMed literature database, we summarize evidence that amino acid oxidation can temporarily compensate for the decreased glucose metabolism, but eventually altered amino acid and amino acid catabolite levels likely lead to toxicities contributing to AD progression. Because amino acids are involved in so many cellular metabolic and signaling pathways, the effects of altered amino acid metabolism in AD brain are far-reaching. Possible pathological results from changes in the levels of several important amino acids are discussed. Urea cycle function may be induced in endothelial cells of AD patient brains, possibly to remove excess ammonia produced from increased amino acid catabolism. Studying AD from a metabolic perspective provides new insights into AD pathogenesis and may lead to the discovery of dietary metabolite supplements that can partially compensate for alterations of enzymatic function to delay AD or alleviate some of the suffering caused by the disease.

Neuroinflammatory paradigms in lysosomal storage diseases

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Megan Elizabeth Bosch

2015-10-01

Full Text Available Lysosomal storage diseases (LSDs include approximately 70 distinct disorders that collectively account for 14% of all inherited metabolic diseases. LSDs are caused by mutations in various enzymes/proteins that disrupt lysosomal function, which impairs macromolecule degradation following endosome-lysosome and phagosome-lysosome fusion and autophagy, ultimately disrupting cellular homeostasis. LSDs are pathologically typified by lysosomal inclusions composed of a heterogeneous mixture of various proteins and lipids that can be found throughout the body. However, in many cases the CNS is dramatically affected, which may result from heightened neuronal vulnerability based on their post-mitotic state. Besides intrinsic neuronal defects, another emerging factor common to many LSDs is neuroinflammation, which may negatively impact neuronal survival and contribute to neurodegeneration. Microglial and astrocyte activation is a hallmark of many LSDs that affect the CNS, which often precedes and predicts regions where eventual neuron loss will occur. However, the timing, intensity, and duration of neuroinflammation may ultimately dictate the impact on CNS homeostasis. For example, a transient inflammatory response following CNS insult/injury can be neuroprotective, as glial cells attempt to remove the insult and provide trophic support to neurons. However, chronic inflammation, as seen in several LSDs, can promote neurodegeneration by creating a neurotoxic environment due to elevated levels of cytokines, chemokines, and pro-apoptotic molecules. Although neuroinflammation has been reported in several LSDs, the cellular basis and mechanisms responsible for eliciting neuroinflammatory pathways are just beginning to be defined. This review highlights the role of neuroinflammation in select LSDs and its potential contribution to neuron loss.

Study on thermal property of lauric–palmitic–stearic acid/vermiculite composite as form-stable phase change material for energy storage

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Nan Zhang

2015-09-01

Full Text Available The form-stable composite phase change material of lauric–palmitic–stearic acid ternary eutectic mixture/vermiculite was prepared by vacuum impregnation method for thermal energy storage. The maximum mass fraction of lauric–palmitic–stearic acid ternary eutectic mixture retained in vermiculite was determined as 50 wt% without melted phase change material seepage from the composite phase change material. Fourier transformation infrared spectroscope and scanning electron microscope were used to characterize the structure and morphology of the prepared lauric–palmitic–stearic acid ternary eutectic mixture/vermiculite form-stable composite phase change material, and the results indicate that lauric–palmitic–stearic acid ternary eutectic mixture was well confined into the layer porous structure of vermiculite by physical reaction. The melting and freezing temperatures and latent heats were measured by differential scanning calorimeter as 31.4°C and 30.3°C, and 75.8 and 73.2 J/g, respectively. Thermal cycling test showed that there was no significant change in the thermal properties of lauric–palmitic–stearic acid ternary eutectic mixture/vermiculite form-stable composite phase change material after 1000 thermal cycles. Moreover, 2 wt% expanded graphite was added to improve the thermal conductivity of lauric–palmitic–stearic acid ternary eutectic mixture/vermiculite form-stable composite phase change material. All results indicated that the prepared lauric–palmitic–stearic acid ternary eutectic mixture/vermiculite form-stable composite phase change material had suitable thermal properties and good thermal reliability for the application of thermal energy storage in building energy efficiency.

Serial changes in plasma K concentration during storage of irradiation blood products

International Nuclear Information System (INIS)

Togashi, Kazue; Yamada, Keiko; Otake, Sachiko; Saito, Yukiko; Sugimura, Kazuhito; Takahashi, Hoyu

1996-01-01

Irradiation of blood products is highly effective in the prevention of transfusion-associated graft-versus-host disease (GVHD). In order to assess the safe storage period of irradiated blood products, serial changes in plasma K, red cell 2,3-diphosphoglycerate (2,3-DPG) and lactic acid concentrations of whole blood and M·A·P-added red cell concentrate (RC-M·A·P) during storage at 5degC were measured after irradiation with 137 Cs by IBL 437C (CIS bio international). Plasma K concentration did not change immediately after irradiation, but increased more rapidly and in a radiation dose-dependent manner in the irradiated products than nonirradiated products. The changes in red cell 2,3-DPG and lactic acid concentrations were not affected by irradiation but were rather dependent on the storage period after blood collection. Plasma K concentrations of whole blood and RC-M·A·P irradiated with 25 Gy increased at 5 and 3 days, respectively, to the K levels observed after the storage of nonirradiated products for 21 days. It is therefore recommended that whole blood be used within 5 days and RC-M·A·P within 3 days when stored after irradiation with 25 Gy. (author)

Excitatory amino acid receptors and disease.

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Meldrum, B S

1992-08-01

Recent advances in the molecular biology of excitatory amino acid receptors are reviewed. Evidence that drugs blocking the excitatory action of glutamate at the N-methyl-D-aspartate (NMDA) and non-NMDA receptors may be of clinical use in epilepsy, Parkinson's disease, cerebral ischaemia and trauma, acquired immune deficiency syndrome (AIDS) encephalopathy and neuropathic pain is summarized.

Mitochondrial dysfunction in human skeletal muscle biopsies of lipid storage disorder.

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Debashree, Bandopadhyay; Kumar, Manish; Keshava Prasad, Thottethodi Subrahmanya; Natarajan, Archana; Christopher, Rita; Nalini, Atchayaram; Bindu, Parayil Sankaran; Gayathri, Narayanappa; Srinivas Bharath, Muchukunte Mukunda

2018-02-09

Mitochondria regulate the balance between lipid metabolism and storage in the skeletal muscle. Altered lipid transport, metabolism and storage influence the bioenergetics, redox status and insulin signalling, contributing to cardiac and neurological diseases. Lipid storage disorders (LSDs) are neurological disorders which entail intramuscular lipid accumulation and impaired mitochondrial bioenergetics in the skeletal muscle causing progressive myopathy with muscle weakness. However, the mitochondrial changes including molecular events associated with impaired lipid storage have not been completely understood in the human skeletal muscle. We carried out morphological and biochemical analysis of mitochondrial function in muscle biopsies of human subjects with LSDs (n = 7), compared to controls (n = 10). Routine histology, enzyme histochemistry and ultrastructural analysis indicated altered muscle cell morphology and mitochondrial structure. Protein profiling of the muscle mitochondria from LSD samples (n = 5) (vs. control, n = 5) by high-throughput mass spectrometric analysis revealed that impaired metabolic processes could contribute to mitochondrial dysfunction and ensuing myopathy in LSDs. We propose that impaired fatty acid and respiratory metabolism along with increased membrane permeability, elevated lipolysis and altered cristae entail mitochondrial dysfunction in LSDs. Some of these mechanisms were unique to LSD apart from others that were common to dystrophic and inflammatory muscle pathologies. Many differentially regulated mitochondrial proteins in LSD are linked with other human diseases, indicating that mitochondrial protection via targeted drugs could be a treatment modality in LSD and related metabolic diseases. © 2018 International Society for Neurochemistry.

Modeling the vacuolar storage of malate shed lights on pre- and post-harvest fruit acidity.

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Etienne, Audrey; Génard, Michel; Lobit, Philippe; Bugaud, Christophe

2014-11-18

Malate is one of the most important organic acids in many fruits and its concentration plays a critical role in organoleptic properties. Several studies suggest that malate accumulation in fruit cells is controlled at the level of vacuolar storage. However, the regulation of vacuolar malate storage throughout fruit development, and the origins of the phenotypic variability of the malate concentration within fruit species remain to be clarified. In the present study, we adapted the mechanistic model of vacuolar storage proposed by Lobit et al. in order to study the accumulation of malate in pre and postharvest fruits. The main adaptation concerned the variation of the free energy of ATP hydrolysis during fruit development. Banana fruit was taken as a reference because it has the particularity of having separate growth and post-harvest ripening stages, during which malate concentration undergoes substantial changes. Moreover, the concentration of malate in banana pulp varies greatly among cultivars which make possible to use the model as a tool to analyze the genotypic variability. The model was calibrated and validated using data sets from three cultivars with contrasting malate accumulation, grown under different fruit loads and potassium supplies, and harvested at different stages. The model predicted the pre and post-harvest dynamics of malate concentration with fairly good accuracy for the three cultivars (mean RRMSE = 0.25-0.42). The sensitivity of the model to parameters and input variables was analyzed. According to the model, vacuolar composition, in particular potassium and organic acid concentrations, had an important effect on malate accumulation. The model suggested that rising temperatures depressed malate accumulation. The model also helped distinguish differences in malate concentration among the three cultivars and between the pre and post-harvest stages by highlighting the probable importance of proton pump activity and particularly of the free

Alterations in membrane trafficking and pathophysiological implications in lysosomal storage disorders.

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Kuech, Eva-Maria; Brogden, Graham; Naim, Hassan Y

2016-11-01

Lysosomal storage disorders are a heterogeneous group of more than 50 distinct inborn metabolic diseases affecting about 1 in 5000 to 7000 live births. The diseases often result from mutations followed by functional deficiencies of enzymes or transporters within the acidic environment of the lysosome, which mediate the degradation of a wide subset of substrates, including glycosphingolipids, glycosaminoglycans, cholesterol, glycogen, oligosaccharides, peptides and glycoproteins, or the export of the respective degradation products from the lysosomes. The progressive accumulation of uncleaved substrates occurs in multiple organs and finally causes a broad spectrum of different pathologies including visceral, neurological, skeletal and hematologic manifestations. Besides deficient lysosomal enzymes and transporters other defects may lead to lysosomal storage disorders, including activator defects, membrane defects or defects in modifier proteins. In this review we concentrate on four different lysosomal storage disorders: Niemann-Pick type C, Fabry disease, Gaucher disease and Pompe disease. While the last three are caused by defective lysosomal hydrolases, Niemann-Pick type C is caused by the inability to export LDL-derived cholesterol out of the lysosome. We want to emphasise potential implications of membrane trafficking defects on the pathology of these diseases, as many mutations interfere with correct lysosomal protein trafficking and alter cellular lipid homeostasis. Current therapeutic strategies are summarised, including substrate reduction therapy as well as pharmacological chaperone therapy which directly aim to improve folding and lysosomal transport of misfolded mutant proteins. Copyright © 2016 Elsevier B.V. and Société Française de Biochimie et Biologie Moléculaire (SFBBM). All rights reserved.

Reprint of: Marine OMEGA-3 fatty acids in the prevention of cardiovascular disease.

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Mori, Trevor A

2018-04-12

Omega-6 (ω6) and omega-3 (ω3) fatty acids are two classes of dietary polyunsaturated fatty acids derived from linoleic acid (18:2ω6) and α-linolenic acid (18:3ω3), respectively. Enzymatic metabolism of linoleic and α-linolenic acids generates arachidonic acid (20:4ω6) and eicosapentaenoic acid (20:5ω3; EPA), respectively, both of which are substrates for enzymes that yield eicosanoids with multiple and varying physiological functions. Further elongation and desaturation of EPA yields the 22-carbon fatty acid docosahexaenoic acid (22:6ω3; DHA). The main dietary source of EPA and DHA for human consumption is fish, especially oily fish. There is considerable evidence that EPA and DHA are protective against cardiovascular disease (heart disease and stroke), particularly in individuals with pre-existing disease. ω3 Fatty acids benefit multiple risk factors including blood pressure, blood vessel function, heart function and blood lipids, and they have antithrombotic, anti-inflammatory and anti-oxidative actions. ω3 Fatty acids do not adversely interact with medications. Supplementation with ω3 fatty acids is recommended in individuals with elevated blood triglyceride levels and patients with coronary heart disease. A practical recommendation for the general population is to increase ω3 fatty acid intake by incorporating fish as part of a healthy diet that includes increased fruits and vegetables, and moderation of salt intake. Health authorities recommend the general population should consume at least two oily fish meals per week. Copyright © 2017 Elsevier B.V. All rights reserved.

Impact of Precooling and Controlled-Atmosphere Storage on γ-Aminobutyric Acid (GABA) Accumulation in Longan (Dimocarpus longan Lour.) Fruit.

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Zhou, Molin; Ndeurumio, Kessy H; Zhao, Lei; Hu, Zhuoyan

2016-08-24

Longan (Dimocarpus longan Lour.) fruit cultivars 'Chuliang' and 'Shixia' were analyzed for γ-aminobutyric acid (GABA) accumulation after precooling and in controlled-atmosphere storage. Fruit were exposed to 5% O2 plus 3%, 5%, or 10% CO2 at 4 °C, and GABA and associated enzymes, aril firmness, and pericarp color were measured. Aril softening and pericarp browning were delayed by 5% CO2 + 5% O2. GABA concentrations and glutamate decarboxylase (GAD; EC 4.1.1.15) activities declined during storage at the higher-CO2 treatments. However, GABA aminotransferase (GABA-T; EC 2.6.1.19) activities in elevated CO2-treated fruit fluctuated during storage. GABA concentrations increased after precooling treatments. GAD activity and GABA-T activity were different between cultivars after precooling. GABA concentrations in fruit increased after 3 days of 10% CO2 + 5% O2 treatment and then declined as storage time increased. GABA accumulation was associated with stimulation of GAD activity rather than inhibition of GABA-T activity.

Omega-3 fatty acids and coronary heart disease. The final verdict?

NARCIS (Netherlands)

Kromhout, D.

2012-01-01

Purpose of review: The fish fatty acids eicosapentenoic acid (EPA) and docosahexenoic acid (DHA) may be protective against fatal coronary heart disease (CHD) and sudden cardiac death. This review summarizes the recent findings of prospective cohort studies and randomized controlled trials. Recent

Fulminant lipid storage myopathy due to multiple acyl-coenzyme a dehydrogenase deficiency.

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Whitaker, Charles H; Felice, Kevin J; Silvers, David; Wu, Qian

2015-08-01

The lipid storage myopathies, primary carnitine deficiency, neutral lipid storage disease, and multiple acyl coenzyme A dehydrogenase deficiency (MADD), are progressive disorders that cause permanent weakness. These disorders of fatty acid metabolism and intracellular triglyceride degradation cause marked fat deposition and damage to muscle cells. We describe a rapidly progressive myopathy in a previously healthy 33-year-old woman. Over 4 months, she developed a proximal and axial myopathy associated with diffuse myalgia and dysphagia, ultimately leading to respiratory failure and death. Muscle biopsy showed massive accumulation of lipid. Plasma acylcarnitine and urine organic acid analysis was consistent with MADD. This was confirmed by molecular genetic testing, which revealed 2 pathogenic mutations in the ETFDH gene. This report illustrates a late-onset case of MADD and reviews the differential diagnosis and evaluation of patients with proximal myopathy and excessive accumulation of lipid on muscle biopsy. © 2014 Wiley Periodicals, Inc.

Thermal energy storage properties of mannitol–fatty acid esters as novel organic solid–liquid phase change materials

International Nuclear Information System (INIS)

Sari, Ahmet

2012-01-01

Highlights: ► Four kinds of mannitol–fatty acid esters were synthesized as novel organic PCMs for thermal energy storage applications. ► The synthesized PCMs were characterized using FT-IR, 1 H NMR, 13 C NMR, DSC and TGA methods and thermal cycling test. ► The melting temperatures and latent heat values of the PCMs were in the range of 42–65 °C and 145–202 J/g, respectively. ► Thermal conductivity of the PCMs was increased significantly by addition of EG with especially 10 wt%. ► The synthesized PCMs are promising organic PCMs for solar heating applications. - Abstract: In this study, four kinds of mannitol–fatty acid esters were synthesized as novel organic phase change materials (PCMs) for thermal energy storage applications. The structural characterization of synthesized mannitol hexastearate (MHS), mannitol hexapalmitate (MHP), mannitol hexamyristate (MHM) and mannitol hexalaurate (MHL) were carried out using Fourier Transform Infrared (FT-IR), Proton Nuclear Magnetic Resonance ( 1 H NMR), and 13 C NMR spectroscopy methods. Thermal energy storage properties and thermal reliability of the synthesized PCMs were determined using differential scanning calorimetry (DSC) method at a heating rate of 1 °C/min. DSC results showed that the melting temperatures of the PCMs were in the temperature range of 42–65 °C and their latent heat values spanned between 145 and 202 J/g. The latent heats of these PCMs are low compared to mannitol but they fall into the same range as fatty acids. The synthesized PCMs have much lower phase change temperatures and supercooling degree (about 1–8 °C) and compared to the mannitol. They have also better odor, noncorrosivity and thermal durability properties as compared to the fatty acids. Thermal cycling test consisted of repeated 1000 melting/solidification cycles also revealed that the synthesized PCMs have good thermal reliability. In addition, thermal conductivity of the PCMs was increased significantly by

Role of non-acid reflux in patients with non-erosive reflux disease

OpenAIRE

Karamanolis, Georgios P.; Tutuian, Radu

2013-01-01

Non-erosive reflux disease (NERD) is the most common presentation of gastroesophageal reflux disease. Although acid reflux is the most important cause of symptom generation in NERD patients, non-acid reflux is also associated with reflux symptoms. The temporal relation between symptoms and reflux episodes is of importance in evaluating the results of combined pH-impedance monitoring in NERD patients. Mucosal hypersensitivity and mechanical stimulation due to great volume of non-acid reflux ar...

Structural consequences of amino acid substitutions causing Tay-Sachs disease.

Science.gov (United States)

Ohno, Kazuki; Saito, Seiji; Sugawara, Kanako; Sakuraba, Hitoshi

2008-08-01

To determine the structural changes in the alpha-subunit of beta-hexosaminidase due to amino acid substitutions causing Tay-Sachs disease, we built structural models of mutant alpha-subunits resulting from 33 missense mutations (24 infantile and 9 late-onset), and analyzed the influence of each amino acid replacement on the structure by calculating the number of atoms affected and determining the solvent-accessible surface area of the corresponding amino acid residue in the wild-type alpha-subunit. In the infantile Tay-Sachs group, the number of atoms influenced by a mutation was generally larger than that in the late-onset Tay-Sachs group in both the main chain and the side chain, and residues associated with the mutations found in the infantile Tay-Sachs group tended to be less solvent-accessible than those in the late-onset Tay-Sachs group. Furthermore, color imaging determined the distribution and degree of the structural changes caused by representative amino acid substitutions, and that there were also differences between the infantile and late-onset Tay-Sachs disease groups. Structural study is useful for elucidating the basis of Tay-Sachs disease.

Fatty acid esters-based composite phase change materials for thermal energy storage in buildings

International Nuclear Information System (INIS)

Sarı, Ahmet; Karaipekli, Ali

2012-01-01

In this study, fatty acid esters-based composite phase change materials (PCMs) for thermal energy storage were prepared by blending erythritol tetrapalmitate (ETP) and erythritol tetrastearate (ETS) with diatomite and expanded perlite (EP). The maximum incorporation percentage for ETP and ETS into diatomite and EP was found to be 57 wt% and 62 wt%, respectively without melted PCM seepage from the composites. The morphologies and compatibilities of the composite PCMs were structurally characterized using scanning electron microscope (SEM) and Fourier transformation infrared (FT–IR) analysis techniques. Thermal energy storage properties of the composite PCMs were determined by differential scanning calorimetry (DSC) analysis. The DSC analyses results indicated that the composite PCMs were good candidates for building applications in terms of their large latent heat values and suitable phase change temperatures. The thermal cycling test including 1000 melting and freezing cycling showed that composite PCMs had good thermal reliability and chemical stability. TG analysis revealed that the composite PCMs had good thermal durability above their working temperature ranges. Moreover, in order to improve the thermal conductivity of the composite PCMs, the expanded graphite (EG) was added to them at different mass fractions (2%, 5%, and 10%). The best results were obtained for the composite PCMs including 5wt% EG content in terms of the increase in thermal conductivity values and the decrease amount in latent heat capacity. The improvement in thermal conductivity values of ETP/Diatomite, ETS/Diatomite, ETP/EP and ETS/EP were found to be about 68%, 57%, 73% and 75%, respectively. Highlights: ► Fatty acid esters-based composite PCMs were prepared by blending ETP and ETS with diatomite and expanded perlite. ► The composite PCMs were characterized by using SEM, FT–IR, DSC and TG analysis methods. ► The DSC results indicated that the composites PCMs had good thermal

Lysosomal exocytosis and lipid storage disorders.

Science.gov (United States)

Samie, Mohammad Ali; Xu, Haoxing

2014-06-01

Lysosomes are acidic compartments in mammalian cells that are primarily responsible for the breakdown of endocytic and autophagic substrates such as membranes, proteins, and lipids into their basic building blocks. Lysosomal storage diseases (LSDs) are a group of metabolic disorders caused by genetic mutations in lysosomal hydrolases required for catabolic degradation, mutations in lysosomal membrane proteins important for catabolite export or membrane trafficking, or mutations in nonlysosomal proteins indirectly affecting these lysosomal functions. A hallmark feature of LSDs is the primary and secondary excessive accumulation of undigested lipids in the lysosome, which causes lysosomal dysfunction and cell death, and subsequently pathological symptoms in various tissues and organs. There are more than 60 types of LSDs, but an effective therapeutic strategy is still lacking for most of them. Several recent in vitro and in vivo studies suggest that induction of lysosomal exocytosis could effectively reduce the accumulation of the storage materials. Meanwhile, the molecular machinery and regulatory mechanisms for lysosomal exocytosis are beginning to be revealed. In this paper, we first discuss these recent developments with the focus on the functional interactions between lipid storage and lysosomal exocytosis. We then discuss whether lysosomal exocytosis can be manipulated to correct lysosomal and cellular dysfunction caused by excessive lipid storage, providing a potentially general therapeutic approach for LSDs. Copyright © 2014 by the American Society for Biochemistry and Molecular Biology, Inc.

Lysosomal exocytosis and lipid storage disorders

Science.gov (United States)

Samie, Mohammad Ali; Xu, Haoxing

2014-01-01

Lysosomes are acidic compartments in mammalian cells that are primarily responsible for the breakdown of endocytic and autophagic substrates such as membranes, proteins, and lipids into their basic building blocks. Lysosomal storage diseases (LSDs) are a group of metabolic disorders caused by genetic mutations in lysosomal hydrolases required for catabolic degradation, mutations in lysosomal membrane proteins important for catabolite export or membrane trafficking, or mutations in nonlysosomal proteins indirectly affecting these lysosomal functions. A hallmark feature of LSDs is the primary and secondary excessive accumulation of undigested lipids in the lysosome, which causes lysosomal dysfunction and cell death, and subsequently pathological symptoms in various tissues and organs. There are more than 60 types of LSDs, but an effective therapeutic strategy is still lacking for most of them. Several recent in vitro and in vivo studies suggest that induction of lysosomal exocytosis could effectively reduce the accumulation of the storage materials. Meanwhile, the molecular machinery and regulatory mechanisms for lysosomal exocytosis are beginning to be revealed. In this paper, we first discuss these recent developments with the focus on the functional interactions between lipid storage and lysosomal exocytosis. We then discuss whether lysosomal exocytosis can be manipulated to correct lysosomal and cellular dysfunction caused by excessive lipid storage, providing a potentially general therapeutic approach for LSDs. PMID:24668941

Influence of Citric Acid on the Pink Color and Characteristics of Sous Vide Processed Chicken Breasts During Chill Storage

Science.gov (United States)

Lim, Ki-Won

2015-01-01

Chicken breast dipped with citric acid (CA) was treated by sous vide processing and stored in a refrigerated state for 0, 3, 6, 9, and 14 d. A non-dipped control group (CON) and three groups dipped in different concentrations of citric acid concentration were analyzed (0.5%, 0.5CIT; 2.0%, 2CIT and 5.0%, 5CIT; w/v). Cooking yield and moisture content increased due to the citric acid. While the redness of the juice and meat in all groups showed significant increase during storage, the redness of the citric acid groups was reduced compared to the control group (pacid reactive substances (TBARS) were generally lower in the citric acid-treated samples than in untreated ones, indicating extended shelf life of the cooked chicken breast dipped in citric acid solution. The shear force of the 2CIT and 5CIT groups was significantly lower (pcitric acid concentrations. PMID:26761885

Glycyrrhizic Acid in the Treatment of Liver Diseases: Literature Review

Directory of Open Access Journals (Sweden)

Jian-yuan Li

2014-01-01

Full Text Available Glycyrrhizic acid (GA is a triterpene glycoside found in the roots of licorice plants (Glycyrrhiza glabra. GA is the most important active ingredient in the licorice root, and possesses a wide range of pharmacological and biological activities. GA coupled with glycyrrhetinic acid and 18-beta-glycyrrhetic acid was developed in China or Japan as an anti-inflammatory, antiviral, and antiallergic drug for liver disease. This review summarizes the current biological activities of GA and its medical applications in liver diseases. The pharmacological actions of GA include inhibition of hepatic apoptosis and necrosis; anti-inflammatory and immune regulatory actions; antiviral effects; and antitumor effects. This paper will be a useful reference for physicians and biologists researching GA and will open the door to novel agents in drug discovery and development from Chinese herbs. With additional research, GA may be more widely used in the treatment of liver diseases or other conditions.

Clinical and biochemical heterogeneity between patients with glycogen storage disease type IA: the added value of CUSUM for metabolic control.

Science.gov (United States)

Peeks, Fabian; Steunenberg, Thomas A H; de Boer, Foekje; Rubio-Gozalbo, M Estela; Williams, Monique; Burghard, Rob; Rajas, Fabienne; Oosterveer, Maaike H; Weinstein, David A; Derks, Terry G J

2017-09-01

To study heterogeneity between patients with glycogen storage disease type Ia (GSD Ia), a rare inherited disorder of carbohydrate metabolism caused by the deficiency of glucose-6-phosphatase (G6Pase). Descriptive retrospective study of longitudinal clinical and biochemical data and long-term complications in 20 GSD Ia patients. We included 11 patients with homozygous G6PC mutations and siblings from four families carrying identical G6PC genotypes. To display subtle variations for repeated triglyceride measurements with respect to time for individual patients, CUSUM-analysis graphs were constructed. Patients with different homozygous G6PC mutations showed important differences in height, BMI, and biochemical parameters (i.e., lactate, uric acid, triglyceride, and cholesterol concentrations). Furthermore, CUSUM-analysis predicts and displays subtle changes in longitudinal blood triglyceride concentrations. Siblings in families also displayed important differences in biochemical parameters (i.e., lactate, uric acid, triglycerides, and cholesterol concentrations) and long-term complications (i.e., liver adenomas, nephropathy, and osteopenia/osteoporosis). Differences between GSD Ia patients reflect large clinical and biochemical heterogeneity. Heterogeneity between GSD Ia patients with homozygous G6PC mutations indicate an important role of the G6PC genotype/mutations. Differences between affected siblings suggest an additional role (genetic and/or environmental) of modifying factors defining the GSD Ia phenotype. CUSUM-analysis can facilitate single-patient monitoring of metabolic control and future application of this method may improve precision medicine for patients both with GSD and remaining inherited metabolic diseases.

Plasma biomarkers of decreased vesicular storage distinguish Parkinson disease with orthostatic hypotension from the parkinsonian form of multiple system atrophy.

Science.gov (United States)

Goldstein, David S; Kopin, Irwin J; Sharabi, Yehonatan; Holmes, Courtney

2015-02-01

Parkinson disease with orthostatic hypotension (PD + OH) and the parkinsonian form of multiple system atrophy (MSA-P) can be difficult to distinguish clinically. Recent studies indicate that PD entails a vesicular storage defect in catecholaminergic neurons. Although cardiac sympathetic neuroimaging by (18)F-dopamine positron emission tomography can identify decreased vesicular storage, this testing is not generally available. We assessed whether plasma biomarkers of a vesicular storage defect can separate PD + OH from MSA-P. We conceptualized that after F-dopamine injection, augmented production of F-dihydroxyphenylacetic acid (F-DOPAC) indicates decreased vesicular storage, and we therefore predicted that arterial plasma F-DOPAC would be elevated in PD + OH but not in MSA-P. We measured arterial plasma F-DOPAC after (18)F-dopamine administration (infused i.v. over 3 min) in patients with PD + OH (N = 12) or MSA-P (N = 21) and in healthy control subjects (N = 26). Peak F-DOPAC:dihydroxyphenylglycol (DHPG) was also calculated to adjust for effects of denervation on F-DOPAC production. Plasma F-DOPAC accumulated rapidly after initiation of (18)F-dopamine infusion. Peak F-DOPAC (5-10 min) in PD + OH averaged three times that in MSA-P (P 300 nCi-kg/cc-mCi, in contrast with 7 of 12 PD + OH patients (χ(2) = 16.6, P < 0.0001). DHPG was lower in PD + OH (3.83 ± 0.36 nmol/L) than in MSA-P (5.20 ± 0.29 nmol/L, P = 0.007). All MSA-P patients had peak F-DOPAC:DHPG < 60, in contrast with 9 of 12 PD + OH patients (χ(2) = 17.5, P < 0.0001). Adjustment of peak F-DOPAC for DHPG increased test sensitivity from 58 to 81% at similar high specificity. After F-dopamine injection, plasma F-DOPAC and F-DOPAC:DHPG distinguish PD + OH from MSA-P.

Preparation and properties of highly conductive palmitic acid/graphene oxide composites as thermal energy storage materials

International Nuclear Information System (INIS)

Mehrali, Mohammad; Latibari, Sara Tahan; Mehrali, Mehdi; Indra Mahlia, Teuku Meurah; Cornelis Metselaar, Hendrik Simon

2013-01-01

PA/GO (palmitic acid/graphene oxide) as PCMs (phase change materials) prepared by vacuum impregnation method, have high thermal conductivity. The GO (graphene oxide) composite was used as supporting material to improve thermal conductivity and shape stabilization of composite PCM (phase change material). SEM (Scanning electronic microscope), FT-IR (Fourier transformation infrared spectroscope) and XRD (X-ray diffractometer) were applied to determine microstructure, chemical structure and crystalloid phase of palmitic acid/GO composites, respectively. DSC (Differential scanning calorimeter) test was done to investigate thermal properties which include melting and solidifying temperatures and latent heat. FT-IR analysis represented that the composite instruction of porous palmitic acid and GO were physical. The temperatures of melting, freezing and latent heats of the composite measured through DSC analysis were 60.45, 60.05 °C, 101.23 and 101.49 kJ/kg, respectively. Thermal cycling test showed that the form-stable composite PCM has good thermal reliability and chemical stability. Thermal conductivity of the composite PCM was improved by more than three times from 0.21 to 1.02. As a result, due to their acceptable thermal properties, good thermal reliability, chemical stability and great thermal conductivities, we can consider the prepared form-stable composites as highly conductive PCMs for thermal energy storage applications. - Highlights: • Novel composite PCM with high thermal conductivity and latent heat storage. • New thermal cycling test for thermal reliability of composite PCMs. • Increasing thermal conductivity of composite PCM with graphene oxide. • Increasing thermal stability of phase change material by adding graphene oxide

Impact of Refrigerated Storage on Quality of Oil from Freshwater Jarko (Wallago attu Fish.

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Nusrat N. Memon

2010-12-01

Full Text Available The effect of refrigerated storage on the quality of freshwater fish oil Jarko (Wallago attu was evaluated by measuring fatty acid profile, free fatty acids (FFA, peroxide value (PV, acid value (AV, sponification value (SV, iodine value (IV and poylene index (PI up to the time period of 120 days. After 120 days storage, mono unsaturated fatty acids (MUFA and polyunsaturated fatty acids (PUFA contents were decreased by 24.89% and 33.70%, respectively. While, saturated fatty acids (SFA content was found to be increased by 26.82%, against the actual value. The change in polyunsaturated fatty acids during refrigerated storage was measured by the PI value. The PI decreased during storage due to lipid oxidation, but remained nearly constant after 90th day of storage. The results of PV, AV and FFA demonstrates that Wallago attu fish oil remained acceptable for consumption for 60 days but eventually exceeded the recommended values after 60 days of refrigerated storage.

Influence of different storage times and temperatures on blood gas and acid-base balance in ovine venous blood.

Science.gov (United States)

Hussein, H A; Aamer, A A

2013-01-01

The present study was designed to investigate the effects of storage temperature and time on blood gas and acid-base balance of ovine venous blood. Ten clinically healthy sheep were used in this study. A total number of 30 blood samples, were divided into three different groups, and were stored in a refrigerator adjusted to +4 ºC (Group I, n = 10), at RT of about 22-25 ºC (Group II, n = 10) and in an incubator adjusted to 37 ºC (Group III, n = 10) for up to 48 h. Blood samples were analysed for blood gas and acid-base indices at 0, 1, 2, 3, 4, 5, 6, 12, 24 and 48 h of storage. In comparison to the baseline value (0), there were significant decreases of blood pH of samples stored at RT and in the incubator after 1 h (ppO2 values were significantly higher for Group I after 2 h and for Groups II and III after 1 h (preference range and it may be of clinical diagnostic use for up to 6 h.

Life-threatening hyperkalemia following zoledronic acid infusion for Paget's disease: a case report

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Naysmith Morag

2011-08-01

Full Text Available Abstract Introduction Zoledronic acid is a highly effective treatment in Paget's disease for persistent bone pain and prevention of further progression of the disease. The commonest electrolyte abnormality is hypocalcemia. To the best of our knowledge this is the first case of hyperkalemia secondary to zoledronic acid to be published in the world literature. The commonest arrhythmia related to zoledronic acid is atrial fibrillation. Case presentation We describe the case of an 80-year-old Caucasian man, with a history of ischemic heart disease, who had an in-hospital cardiac arrest related to hyperkalemia. Increasing potassium levels were noted following his first zoledronic acid infusion for symptomatic control of bone pain secondary to Paget's disease. Our patient suffered a cardiac arrest 10 days following the zoledronic acid infusion. Our patient's biochemistry and electrocardiogram output were monitored until his death 26 days after his cardiac arrest. Our patient developed paroxysmal atrial fibrillation in the post-resuscitation period and there was persistent hyperkalemia that required prolonged treatment with calcium resonium. All other possible causes of hyperkalemia were excluded. Conclusion In our patient's case persistent hyperkalemia and life-threatening arrhythmias were associated with use of zoledronic acid. These side effects have not been reported before and the causative mechanism is far from clear as there are no obvious systemic effects of zoledronic acid. The combination of zoledronic acid with predisposing factors such as structural heart disease might account for the clinical picture we witnessed. As a result, electrolyte monitoring should be adopted early in zoledronic acid use. Further studies are required to elucidate the underlying mechanism of hyperkalemia and identify the target group of patients where zoledronic acid can be safely administered. Great caution is advised in patients with underlying heart conditions.

Does Altered Uric Acid Metabolism Contribute to Diabetic Kidney Disease Pathophysiology?

Science.gov (United States)

Gul, Ambreen; Zager, Philip

2018-03-01

Multiple experimental and clinical studies have identified pathways by which uric acid may facilitate the development and progression of chronic kidney disease (CKD) in people with diabetes. However, it remains uncertain if the association of uric acid with CKD represents a pathogenic effect or merely reflects renal impairment. In contrast to many published reports, a recent Mendelian randomization study did not identify a causal link between uric acid and CKD in people with type 1 diabetes. Two recent multicenter randomized control trials, Preventing Early Renal Function Loss in Diabetes (PERL) and FEbuxostat versus placebo rAndomized controlled Trial regarding reduced renal function in patients with Hyperuricemia complicated by chRonic kidney disease stage 3 (FEATHER), were recently designed to assess if uric acid lowering slows progression of CKD. We review the evidence supporting a role for uric acid in the pathogenesis of CKD in people with diabetes and the putative benefits of uric acid lowering.

Changes in the carbohydrate and organic acid contents of tomatoes as a function of radiation dose and storage time

Energy Technology Data Exchange (ETDEWEB)

Kovacs, E [Kozponti Elelmiszeripari Kutato Intezet, Budapest (Hungary)

1974-01-01

Sugars and organic acids of tomatoes, variety Original Fruehrot, in different stages of development were examined by gas chromatography after silylation. Changes in the carbohydrate and organic acid content of half-ripe and ripe tomatoes were determined after treatment with different radiation doses (5, 15, 30, 60, 250 and 500 krad). Fructose, glucose, saccharose and malic acid could be detected in the tomato samples. The saccharose and malic acid contents of half-ripe tomatoes decreased as a result of irradiation with doses above 250 krad. In ripe tomatoes, the malic acid content fell most markedly under the influence of irradiation. The sugar content tended to decrease as a function of storage time, except for samples treated with 15 krad which usually delayed decomposition of the components tested. The effect of irradiation was more extensive in half-ripe tomatoes than in ripe ones.

Comparative Study of Stearic Acid/Iron-Oxide Binary and Stearic Acid/Iron-Oxide/Titanium-Oxide Ternary for Use as Energy Storage Material

International Nuclear Information System (INIS)

Andiarto, Rizky; Nuryadin, Muhammad Khalish; Saleh, Rosari

2016-01-01

In this work, a series of stearic acid/Fe 3 O 4 , and stearic acid/Fe 3 O 4 /TiO 2 nanocomposites for thermal energy storage (TES) system were synthesized through a two-step process. Fe 3 O 4 nanoparticles and Fe 3 O 4 /TiO 2 nanocomposites were first prepared using sol-gel methods and then both samples were mixed into stearic acid by dispersion technique at three different weight % ratio to stearic acid: 5%, 10% and 15% to obtain stearic acid/Fe 3 O 4 , and stearic acid/Fe 3 O 4 /TiO 2 nanocomposites. Morphologies and structural properties of the samples were characterized by X-ray diffractometer (XRD), Fourier transform infrared spectroscopy (FTIR), field emission scanning electron microscope (FESEM) and energy dispersive X-ray spectroscopy (EDX), while thermal properties of the sample were determined by differential scanning calorimetry (DSC) and thermogravimetric analysis (TGA). The XRD patterns demonstrate, that stearic acid/Fe 3 O 4 contained characteristic peaks of Fe 3 O 4 and stearic acid structures, while peaks corresponded to anatase TiO 2 structures appear in stearic acid/ Fe 3 O 4 /TiO 2 nanocomposites. From the DSC measurements, it is found that the maximum latent heat was found at samples with weight ratio of 5%. Moreover, the enhancement up to 20% of latent heat in solidifying as well as melting processes was observed. TGA measurements show high degradation temperature in the range of 246 - 251°C. The TGA results also shows that the residual mass of the sample matches the composition of Fe 3 O 4 and Fe 3 O 4 /TiO 2 which is added to the stearic acid. (paper)

A convenient diagnostic function test of peripheral blood neutrophils in glycogen storage disease type Ib

NARCIS (Netherlands)

Verhoeven, A.J.; Visser, G; Van Zwieten, R; Gruszczynska, B; Poll-The, DWEET; Smit, GPA

Neutrophils from patients suffering from glycogen storage disease type To (GSD-Ib) show several defects, one of which is a decreased rate of glucose utilization. In this study, we established experimental conditions to show the stimulation of the neutrophil respiratory burst by extracellular

Effect of chitosan enriched with lycopene coating on fatty acid profile and fat oxidation parameters of rainbow trout fillet during refrigerated storage(orginal reserch article

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Seyede Samane Naghibi

2016-05-01

Full Text Available Fish are the major dietary source of the polyunsaturated fatty acid for humans. Therefore, protection of fish against all types of oxidative corruption seems to be necessary. Lycopene is the source of natural antioxidant. The present study was conducted to evaluate antioxidant properties of lycopene (using the method of DPPH and the combined effect of its various doses (1.5 and 3% and chitosan on fat oxidation parameters and fatty acids composition of Rainbow trout fillet. The analysis was performed after 0, 8 and 16 days of storage of the samples at 4°C to determine peroxide value (PV and free fatty acid content (FFA. In addition, fatty acid compositions was determined by Gas chromatography assay. In control treatment, the fatty acid composition of Rainbow trout fillet was consisted of %20.6±0.03 saturated fatty acids (SFA, %43.81±0.04 monounsaturated fatty acid (MUFA and %32.83±0.03 polyunsaturated fatty acids (PUFAs in 0 day. Statistical analysis showed that there were fewer changes in PV, FFA and proportion of fatty acids between chitosan and lycopene-chitosan treatment in regard to control sample during 16 days of refrigeration storage. Chitosan coated samples enriched with lycopene exhibited less rapidly lipid damages than all the other samples (p

Combination processes for improving storage of irradiated potatoes

International Nuclear Information System (INIS)

Shirsat, S.G.; Thomas, P.; Nair, P.M.

1990-01-01

Various combined treatments of harvested potatoes to reduce microbial spoilage of irradiated potatoes in storage were studied. Treatments studied were irradiation in conjunction with hot water dip, cold or hot salicylic acid dip, dusting with salicylic acid powder, and sodium hypochlorite wash followed by storage in well aerated containers. Microbial spoilage in irradiated potatoes was found to be greatly reduced by storage at 10deg or 15degC in well aerated containers. (M.G.B.). 9 refs., 1 tab

Therapeutic potential of n-3 polyunsaturated fatty acids in disease.

Science.gov (United States)

Fetterman, James W; Zdanowicz, Martin M

2009-07-01

The potential therapeutic benefits of supplementation with n-3 polyunsaturated fatty acids (PUFAs) in various diseases are reviewed, and the antiinflammatory actions, activity, and potential drug interactions and adverse effects of n-3 PUFAs are discussed. Fish oils are an excellent source of long-chain n-3 PUFAs, such as eicosapentaenoic acid and docosahexaenoic acid. After consumption, n-3 PUFAs can be incorporated into cell membranes and reduce the amount of arachidonic acid available for the synthesis of proinflammatory eicosanoids (e.g., prostaglandins, leukotrienes). Likewise, n-3 PUFAs can also reduce the production of inflammatory cytokines, such as tumor necrosis factor alpha, interleukin-1, and interleukin-6. Considerable research has been conducted to evaluate the potential therapeutic effects of fish oils in numerous conditions, including arthritis, coronary artery disease, inflammatory bowel disease, asthma, and sepsis, all of which have inflammation as a key component of their pathology. Additional investigations into the use of supplementation with fish oils in patients with neural injury, cancer, ocular diseases, and critical illness have recently been conducted. The most commonly reported adverse effects of fish oil supplements are a fishy aftertaste and gastrointestinal upset. When recommending an n-3 PUFA, clinicians should be aware of any possible adverse effect or drug interaction that, although not necessarily clinically significant, may occur, especially for patients who may be susceptible to increased bleeding (e.g., patients taking warfarin). The n-3 PUFAs have been shown to be efficacious in treating and preventing various diseases. The wide variation in dosages and formulations used in studies makes it difficult to recommend dosages for specific treatment goals.

Muscle MRI in neutral lipid storage disease (NLSD).

Science.gov (United States)

Garibaldi, Matteo; Tasca, Giorgio; Diaz-Manera, Jordi; Ottaviani, Pierfancesco; Laschena, Francesco; Pantoli, Donatella; Gerevini, Simonetta; Fiorillo, Chiara; Maggi, Lorenzo; Tasca, Elisabetta; D'Amico, Adele; Musumeci, Olimpia; Toscano, Antonio; Bruno, Claudio; Massa, Roberto; Angelini, Corrado; Bertini, Enrico; Antonini, Giovanni; Pennisi, Elena Maria

2017-07-01

Neutral lipid storage disease (NLSD) is a rare inherited disorder of lipid metabolism resulting in lipid droplets accumulation in different tissues. Skeletal muscle could be affected in both two different form of disease: NLSD with myopathy (NLSD-M) and NLSD with ichthyosis (NLSD-I). We present the muscle imaging data of 12 patients from the Italian Network for NLSD: ten patients presenting NLSD-M and two patients with NLSD-I. In NLSD-M gluteus minimus, semimembranosus, soleus and gastrocnemius medialis in the lower limbs and infraspinatus in the upper limbs were the most affected muscles. Gracilis, sartorius, subscapularis, pectoralis, triceps brachii and sternocleidomastoid were spared. Muscle involvement was not homogenous and characteristic "patchy" replacement was observed in at least one muscle in all the patients. Half of the patients showed one or more STIR positive muscles. In both NLSD-I cases muscle involvement was not observed by T1-TSE sequences, but one of them showed positive STIR images in more than one muscle in the leg. Our data provides evidence that muscle imaging can identify characteristic alterations in NLSD-M, characterized by a specific pattern of muscle involvement with "patchy" areas of fatty replacement. Larger cohorts are needed to assess if a distinct pattern of muscle involvement exists also for NLSD-I.

Survival, Quality of Life and Effects of Enzyme Replacement Therapy in Adults with Pompe Disease

NARCIS (Netherlands)

D. Güngör (Deniz)

2013-01-01

textabstractPompe disease, or glycogen storage disorder type II, is a rare inherited metabolic disorder caused by deficiency of the lysosomal enzyme acid α-glucosidase. This results in accumulation of glycogen in cells throughout the body, particularly muscle cells. The disease presents

Neonatal screening for four lysosomal storage diseases with a digital microfluidics platform: Initial results in Brazil

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Eurico Camargo Neto

2018-06-01

Full Text Available Abstract We describe the initial results of a neonatal screening program for four lysosomal storage diseases (MPS I, Pompe, Gaucher and Fabry using the digital microfluidics methodology. The method successfully identified patients previously diagnosed with these diseases and was used to test dried blood spot samples obtained from 10,527 newborns aged 2 to 14 days. The digital microfluidic technology shows potential for a simple, rapid and high-throughput screening for these four diseases in a standard neonatal screening laboratory.

Low brain ascorbic acid increases susceptibility to seizures in mouse models of decreased brain ascorbic acid transport and Alzheimer's disease.

Science.gov (United States)

Warner, Timothy A; Kang, Jing-Qiong; Kennard, John A; Harrison, Fiona E

2015-02-01

Seizures are a known co-occurring symptom of Alzheimer's disease, and they can accelerate cognitive and neuropathological dysfunction. Sub-optimal vitamin C (ascorbic acid) deficiency, that is low levels that do not lead the sufferer to present with clinical signs of scurvy (e.g. lethargy, hemorrhage, hyperkeratosis), are easily obtainable with insufficient dietary intake, and may contribute to the oxidative stress environment of both Alzheimer's disease and epilepsy. The purpose of this study was to test whether mice that have diminished brain ascorbic acid in addition to carrying human Alzheimer's disease mutations in the amyloid precursor protein (APP) and presenilin 1 (PSEN1) genes, had altered electrical activity in the brain (electroencephalography; EEG), and were more susceptible to pharmacologically induced seizures. Brain ascorbic acid was decreased in APP/PSEN1 mice by crossing them with sodium vitamin C transporter 2 (SVCT2) heterozygous knockout mice. These mice have an approximately 30% decrease in brain ascorbic acid due to lower levels of SVCT2 that supplies the brain with ASC. SVCT2+/-APP/PSEN1 mice had decreased ascorbic acid and increased oxidative stress in brain, increased mortality, faster seizure onset latency following treatment with kainic acid (10 mg/kg i.p.), and more ictal events following pentylenetetrazol (50 mg/kg i.p.) treatment. Furthermore, we report the entirely novel phenomenon that ascorbic acid deficiency alone increased the severity of kainic acid- and pentylenetetrazol-induced seizures. These data suggest that avoiding ascorbic acid deficiency may be particularly important in populations at increased risk for epilepsy and seizures, such as Alzheimer's disease. Copyright © 2014 Elsevier B.V. All rights reserved.

Form-Stable Phase Change Materials Based on Eutectic Mixture of Tetradecanol and Fatty Acids for Building Energy Storage: Preparation and Performance Analysis

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Yiran li

2013-10-01

Full Text Available This paper is focused on preparation and performance analysis of a series of form-stable phase change materials (FSPCMs, based on eutectic mixtures as phase change materials (PCMs for thermal energy storage and high-density polyethylene (HDPE-ethylene-vinyl acetate (EVA polymer as supporting materials. The PCMs were eutectic mixtures of tetradecanol (TD–capric acid (CA, TD–lauric acid (LA, and TD–myristic acid (MA, which were rarely explored before. Thermal properties of eutectic mixtures and FSPCMs were measured by differential scanning calorimeter (DSC. The onset melting/solidification temperatures of form-stable PCMs were 19.13 °C/13.32 °C (FS TD–CA PCM, 24.53 °C/24.92 °C (FS TD–LA PCM, and 33.15 °C/30.72 °C (FS TD–MA PCM, respectively, and latent heats were almost greater than 90 J/g. The surface morphologies and chemical stability of form-stable PCM were surveyed by scanning electron microscopy (SEM and Fourier-transform infrared (FT-IR spectroscopy, respectively. The thermal cycling test revealed that the thermal reliability of these three form-stable PCMs was good. Thermal storage/release experiment indicated melting/solidification time was shortened by introducing 10 wt % aluminum powder (AP. It is concluded that these FSPCMs can act as potential building thermal storage materials in terms of their satisfactory thermal properties.

Form-Stable Phase Change Materials Based on Eutectic Mixture of Tetradecanol and Fatty Acids for Building Energy Storage: Preparation and Performance Analysis.

Science.gov (United States)

Huang, Jingyu; Lu, Shilei; Kong, Xiangfei; Liu, Shangbao; Li, Yiran

2013-10-22

This paper is focused on preparation and performance analysis of a series of form-stable phase change materials (FSPCMs), based on eutectic mixtures as phase change materials (PCMs) for thermal energy storage and high-density polyethylene (HDPE)-ethylene-vinyl acetate (EVA) polymer as supporting materials. The PCMs were eutectic mixtures of tetradecanol (TD)-capric acid (CA), TD-lauric acid (LA), and TD-myristic acid (MA), which were rarely explored before. Thermal properties of eutectic mixtures and FSPCMs were measured by differential scanning calorimeter (DSC). The onset melting/solidification temperatures of form-stable PCMs were 19.13 °C/13.32 °C (FS TD-CA PCM), 24.53 °C/24.92 °C (FS TD-LA PCM), and 33.15 °C/30.72 °C (FS TD-MA PCM), respectively, and latent heats were almost greater than 90 J/g. The surface morphologies and chemical stability of form-stable PCM were surveyed by scanning electron microscopy (SEM) and Fourier-transform infrared (FT-IR) spectroscopy, respectively. The thermal cycling test revealed that the thermal reliability of these three form-stable PCMs was good. Thermal storage/release experiment indicated melting/solidification time was shortened by introducing 10 wt % aluminum powder (AP). It is concluded that these FSPCMs can act as potential building thermal storage materials in terms of their satisfactory thermal properties.

Phosphorus effects on the mycelium and storage structures of an arbuscular mycorrhizal fungus as studied in the soil and roots by analysis of fatty acid signatures

DEFF Research Database (Denmark)

Olsson, P.A.; Bååth, E.; Jakobsen, I.

1997-01-01

The distribution of an arbuscular mycorrhizal (AM) fungus between soil and roots, and between mycelial and storage structures, was studied by use of the fatty acid signature 16:1 omega 5. Increasing the soil phosphorus level resulted in a decrease in the level of the fatty acid 16:1 omega 5...... in the soil and roots. A similar decrease was detected by microscopic measurements of root colonization and of the length of AM fungal hyphae in the soil. The fatty acid 16:1 omega 5 was estimated from two types of lipids, phospholipids and neutral lipids, which mainly represent membrane lipids and storage...... lipids, respectively. The numbers of spores of the AM fungus formed in the soil correlated most closely, with neutral lipid fatty acid 16:1 omega 5, whereas the hyphal length in the soil correlated most closely with phospholipid fatty acid 16:1 omega 5. The fungal neutral lipid/phospholipid ratio...

Association of plasma uric acid with ischaemic heart disease and blood pressure

DEFF Research Database (Denmark)

Palmer, Tom M; Nordestgaard, Børge G; Benn, Marianne

2013-01-01

To assess the associations between both uric acid levels and hyperuricaemia, with ischaemic heart disease and blood pressure, and to explore the potentially confounding role of body mass index.......To assess the associations between both uric acid levels and hyperuricaemia, with ischaemic heart disease and blood pressure, and to explore the potentially confounding role of body mass index....

Apoptosis and signalling in acid sphingomyelinase deficient cells

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Sillence Dan J

2001-11-01

Full Text Available Abstract Background Recent evidence suggests that the activation of a non-specific lipid scramblase during apoptosis induces the flipping of sphingomyelin from the cell surface to the cytoplasmic leaftet of the plasma membrane. Inner leaflet sphingomyelin is then cleaved to ceramide by a neutral sphingomyelinase. The production of this non-membrane forming lipid induces blebbing of the plasma membrane to aid rapid engulfment by professional phagocytes. However contrary evidence suggests that cells which are deficient in acid sphingomyelinase are defective in apoptosis signalling. This data has been interpreted as support for the activation of acid sphingomyelinase as an early signal in apoptosis. Hypothesis An alternative explanation is put forward whereby the accumulation of intracellular sphingomyelin in sphingomyelinase deficient cells leads to the formation of intracellular rafts which lead to the sequestration of important signalling molecules that are normally present on the cell surface where they perform their function. Testing the hypothesis It is expected that the subcellular distribution of important signalling molecules is altered in acid sphingomyelinase deficient cells, leading to their sequestration in late endosomes / lysosomes. Other sphingolipid storage diseases such as Niemann-Pick type C which have normal acid sphingomyelinase activity would also be expected to show the same phenotype. Implications of the hypothesis If true the hypothesis would provide a mechanism for the pathology of the sphingolipid storage diseases at the cellular level and also have implications for the role of ceramide in apoptosis.

Insights into the structural biology of Gaucher disease.

Science.gov (United States)

Smith, Laura; Mullin, Stephen; Schapira, Anthony H V

2017-12-01

Gaucher disease, the most common lysosomal storage disorder, is caused by mutations in the gene encoding the acid-β-glucosidase lysosomal hydrolase enzyme that cleaves glucocerebroside into glucose and ceramide. Reduced enzyme activity and impaired structural stability arise due to >300 known disease-causing mutations. Several of these mutations have also been associated with an increased risk of Parkinson disease (PD). Since the discovery of the acid-β-glucosidase X-ray structure, there have been major advances in our understanding of the structural properties of the protein. Analysis of specific residues has provided insight into their functional and structural importance and provided insight into the pathogenesis of Gaucher disease and the contribution to PD. Disease-causing mutations are positioned throughout the acid-β-glucosidase structure, with many located far from the active site and thus retaining some enzymatic activity however, thus far no clear relationship between mutation location and disease severity has been established. Here, we review the crystal structure of acid-β-glucosidase, while highlighting important structural aspects of the protein in detail. This review discusses the structural stability of acid-β-glucosidase, which can be altered by pH and glycosylation, and explores the relationship between known Gaucher disease and PD mutations, structural stability and disease severity. Copyright © 2017. Published by Elsevier Inc.

INHIBITION OF STAPHYLOCOCCUS AUREUS BY LACTIC ACID BACTERIA AND / OR BIFIDOBACTERIUM LACTIS DURING MILK FERMENTATION AND STORAGE

Directory of Open Access Journals (Sweden)

Khalaf S. Al-Delaimy

2013-02-01

Full Text Available Survival and inhibition of Staphylococcus aureus by the lactic acid bacteria (LAB starter culture (Sterptococcus thermophillus and Lactobacillus delbrukii subsp. bulgaricus and/ or probiotic bacteria Bifidobacterium lactis during milk fermentation to yoghurt and storage up to 12 days was studied. Adding S. aureus (initial count log 6.64/ ml with LAB (initial count log 6.8/ ml in milk during yoghurt processing and storage resulted in no significant change in the counts of both S. aureus and LAB during fermentation period of 4 hrs at 45° C. A steady decrease in S. aureus count during storage at 25° C and 4° C was observed reaching a complete (100 % inhibition after 9 and 12 days, respectively, with no significant increase in LAB count. Adding S. aureus (initial count log 6.62/ ml with B. lactis (initial count log 6.83/ ml in milk for 4 hr at45° C, no significant changes in the counts of both bacteria were found. After storage at 25° C and at 4° C a sharp decline in the S. aureus count with a 100 % inhibition after 6 and 9 days with approximately two log and one log increase in B. lactis counts consecutively. In general similar result was observed when adding S. aureus together with LAB and B. lactis in milk during fermentation and storage. pH values decreased during milk fermentation and storage from initially 6.55-6.64 to around 4 in most milk samples. The results of this study show that S. aureus was completely inhibited by LAB and/or B. lactis after milk fermentation to yoghurt and storage at room temperature and refrigeration for 6-9 days. It is therefore recommended to add the probiotic B. lactis with LAB to milk for yoghurt processing.

Flexible graphene/carbon nanotube hybrid papers chemical-reduction-tailored by gallic acid for high-performance electrochemical capacitive energy storages

Science.gov (United States)

Yao, Lu; Zhou, Chao; Hu, Nantao; Hu, Jing; Hong, Min; Zhang, Liying; Zhang, Yafei

2018-03-01

Mechanically robust graphene papers with both high gravimetric and volumetric capacitances are desired for high-performance energy storages. However, it's still a challenge to tailor the structure of graphene papers in order to meet this requirement. In this work, a kind of chemical-reduction-tailored mechanically-robust reduced graphene oxide/carbon nanotube hybrid paper has been reported for high-performance electrochemical capacitive energy storages. Gallic acid (GA), as an excellent reducing agent, was used to reduce graphene oxide. Through vacuum filtration of gallic acid reduced graphene oxide (GA-rGO) and carboxylic multiwalled carbon nanotubes (MWCNTs) aqueous suspensions, mechanically robust GA-rGO/MWCNTs hybrid papers were obtained. The resultant hybrid papers showed high gravimetric capacitance of 337.6 F g-1 (0.5 A g-1) and volumetric capacitance of 151.2 F cm-3 (0.25 A cm-3). In addition, the assembled symmetric device based on the hybrid papers exhibited high gravimetric capacitance of 291.6 F g-1 (0.5 A g-1) and volumetric capacitance of 136.6 F cm-3 (0.25 A cm-3). Meanwhile, it exhibited excellent rate capability and cycling stability. Above all, this chemical reduction tailoring technique and the resultant high-performance GA-rGO/MWCNTs hybrid papers give an insight for designing high-performance electrodes and hold a great potential in the field of energy storages.

Ursodeoxycholic acid for cystic fibrosis-related liver disease.

Science.gov (United States)

Cheng, Katharine; Ashby, Deborah; Smyth, Rosalind L

2017-09-11

Abnormal biliary secretion leads to the thickening of bile and the formation of plugs within the bile ducts; the consequent obstruction and abnormal bile flow ultimately results in the development of cystic fibrosis-related liver disease. This condition peaks in adolescence with up to 20% of adolescents with cystic fibrosis developing chronic liver disease. Early changes in the liver may ultimately result in end-stage liver disease with people needing transplantation. One therapeutic option currently used is ursodeoxycholic acid. This is an update of a previous review. To analyse evidence that ursodeoxycholic acid improves indices of liver function, reduces the risk of developing chronic liver disease and improves outcomes in general in cystic fibrosis. We searched the Cochrane CF and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches, handsearches of relevant journals and abstract books of conference proceedings. We also contacted drug companies and searched online trial registries.Date of the most recent search of the Group's trials register: 09 April 2017. Randomised controlled trials of the use of ursodeoxycholic acid for at least three months compared with placebo or no additional treatment in people with cystic fibrosis. Two authors independently assessed trial eligibility and quality. The authors used GRADE to assess the quality of the evidence. Twelve trials have been identified, of which four trials involving 137 participants were included; data were only available from three of the trials (118 participants) since one cross-over trial did not report appropriate data. The dose of ursodeoxycholic acid ranged from 10 to 20 mg/kg/day for up to 12 months. The complex design used in two trials meant that data could only be analysed for subsets of participants. There was no significant difference in weight change, mean difference -0.90 kg (95% confidence interval -1.94 to 0.14) based on 30

Chlorogenic Acid and Mental Diseases: From Chemistry to Medicine.

Science.gov (United States)

Nabavi, Seyed Fazel; Tejada, Silvia; Setzer, William N; Gortzi, Olga; Sureda, Antoni; Braidy, Nady; Daglia, Maria; Manayi, Azadeh; Nabavi, Seyed Mohammad

2017-01-01

At present, much attention has been focused on the beneficial effects of natural products on the human health due to their high efficacy and low adverse effects. Among them, polyphenolic compounds are known as one of the most important and common classes of natural products, which possess multiple range of health-promotion effects including anti-inflammatory and antioxidant activities. A plethora of scientific evidence has shown that polyphenolic compounds possess beneficial effects on the central nervous system. Data were collected from Web of Science (ISI Web of Knowledge), Medline, Pubmed, Scopus, Embase, and BIOSIS Previews (from 1950 to 2015), through searching of these keywords: "chlorogenic acid and mental diseases" and "chlorogenic acid and neuroprotection". Chlorogenic acid is known as one of the most common polyphenolic compounds, and is found in different types of fruits and vegetables, spices, wine, olive oil, as well as coffee. The potential neuroprotective effects of chlorogenic acid have been highlighted in several in vitro and in vivo studies. This review critically analyses the available scientific evidence regarding the neuroprotective effects of chlorogenic acid, and its neuropharmacological mechanisms of action. In addition, we also discuss its biosynthesis, sources, bioavailability and metabolism, to provide a broad perspective of the therapeutic implications of this compound in brain health and disease. The present review showed that chlorogenic acid possesses neuroprotective effects under the both in vitro and in vivo models. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

Effect of dietary fiber on serum bile acids in patients with chronic cholestatic liver disease under ursodeoxycholic acid therapy

NARCIS (Netherlands)

Sauter, G.; Beuers, U.; Paumgartner, G.

1995-01-01

During ursodeoxycholic acid therapy for chronic cholestatic liver disease, the serum levels of lithocholic acid increase about twofold. Lithocholic acid has been shown to be hepatotoxic in some animal species. Administration of psyllium hydrophilic mucilloid (PHM), a dietary fiber, has been reported

Could be serum uric acid a risk factor for thrombosis and/or uveitis in Behcet's disease?

Science.gov (United States)

Atıl, Avcı; Deniz, Avcı

2018-01-01

Introduction Serum uric acid level increases in many inflammatory conditions. Uric acid triggers the vascular inflammation and artery damage, which causes to an increased risk of endothelial dysfunction and atherosclerosis. It is not clear in the literature whether uric acid contributes to uveitis by similar mechanisms. We investigated whether uric acid level increases in Behcet's disease patients with thrombosis or anterior uveitis. Patients and methods We reviewed the medical records of 914 Behcet's disease. After screening for exclusion criteria, there were 50 Behcet's disease patients with thrombotic complication and as the control group 202 BD patients without any vascular complication were included to the study. In the Anterior uveitis group, there were 53 Behcet's disease patients. The Control group consisted of 39 Behçet's disease patients without eye findings. Results Mean uric acid value was 4.96 ± 1.06 mg/dl in Behcet's disease patients with thrombosis whereas 4.08 ± 0.94 mg/dl in controls, indicating a significant difference ( p uric acid level was greater in Behcet's disease patients that have a thrombotic complication compared to those without thrombotic complication. Uric acid seems to play a role in the pathogenesis of thrombosis. It is concluded that the elevation of serum uric acid level in patients with anterior uveitis with Behcet's disease is not statistically significant.

Influence of the addition and storage time of crude extract of tea leaves (camellia sinensis l.) toward value of free fatty acid in crude palm oil

Science.gov (United States)

Erwin; Wahifiyah, E.; Hairani, R.; Panggabean, A. S.

2018-04-01

The purpose of this study was to determine the effect of the crude extract of tea leaves (Camellia sinensis L.) and storage time on the content of free fatty acid in palm oil. The dried tea leaves were macerated and concentrated by rotary evaporator. The extract obtained was added to crude palm oil with various added mass of the extract and various storage times. Phytochemical tests indicated the presence of secondary metabolites including alkaloids, triterpenoids, steroids, phenolics and flavonoids. The ANOVA test showed a decrease in free fatty acid content in crude palm oil with the addition of tea leaves extract. The LSD (Least Significant Difference) test showed the best influence on ALB of palm oil is on the total extract mass of 2 grams and the storage time of 20 days.

Changes in the carbohydrate and organic acid contents of tomatoes as a function of radiation dose and storage time

International Nuclear Information System (INIS)

Kovacs, E.

1974-01-01

Sugars and organic acids of tomatoes, variety Original Fruehrot, in different stages of development were examined by gas chromatography after silylation. Changes in the carbohydrate and organic acid content of half-ripe and ripe tomatoes were determined after treatment with different radiation doses (5, 15, 30, 60, 250 and 500 krad). Fructose, glucose, saccharose and malic acid could be detected in the tomato samples. The saccharose and malic acid contents of half-ripe tomatoes decreased as a result of irradiation with doses above 250 krad. In ripe tomatoes, the malic acid content fell most markedly under the influence of irradiation. The sugar content tended to decrease as a function of storage time, except for samples treated with 15 krad which usually delayed decomposition of the components tested. The effect of irradiation was more extensive in half-ripe tomatoes than in ripe ones. (F.J.)

Stearic-acid/carbon-nanotube composites with tailored shape-stabilized phase transitions and light–heat conversion for thermal energy storage

International Nuclear Information System (INIS)

Li, Benxia; Nie, Shibin; Hao, Yonggan; Liu, Tongxuan; Zhu, Jinbo; Yan, Shilong

2015-01-01

Highlights: • A facile preparation of shape-stabilized composite PCMs for thermal energy storage. • The composite PCMs present tunable phase change temperatures and enthalpy. • Sunlight-driven phase change for photothermal conversion and storage. - Abstract: The development of functional materials with both light–heat conversion and thermal energy storage properties is of crucial importance for efficient utilization of sunlight to meet the growing demand for sustainable energy. In this work, the shape-stabilized phase change composites were designed and prepared by integration of stearic acid (SA) and acid-treated carbon nanotubes (a-CNTs). The a-CNTs not only acted as a flexible matrix but also endowed the composites high light–heat conversion ability. The reversible phase transitions shifted from high temperatures (T m = 74 °C, T f = 57 °C) of pure SA to near room temperature (T m = ∼30 °C, T f = ∼22 °C) of SA/a-CNTs composites, probably resulting from the strong interface confinement effect. The phase change enthalpy of the SA/a-CNTs composite could also be tailored by changing the mass ratio of SA and a-CNTs. The composites containing SA of 54.2 wt.%, 67.8 wt.% and 79.5 wt.% presented the melting enthalpy of 76.3 J/g, 98.8 J/g and 111.8 J/g, respectively. Moreover, the phase transition of SA/a-CNTs composite could be driven by sunlight for the energy storage/release. Therefore, this research provides a new platform for improving solar utilization, and understanding the phase transition behaviors of organic PCMs in dimensionally confined environments as well

Targeted modification of storage protein content resulting in improved amino acid composition of barley grain

DEFF Research Database (Denmark)

Sikdar, Md. Shafiqul Islam; Bowra, S; Schmidt, Daiana

2016-01-01

family members. Analysis of the AA composition of the transgenic lines showed that the level of essential amino acids increased with a concomitant reduction in proline and glutamine. Both the barley C-hordein and wheat ω-gliadin genes proved successful for RNAi-gene mediated suppression of barley C......C-hordein in barley and ω-gliadins in wheat are members of the prolamins protein families. Prolamins are the major component of cereal storage proteins and composed of non-essential amino acids (AA) such as proline and glutamine therefore have low nutritional value. Using double stranded RNAi...... silencing technology directed towards C-hordein we obtained transgenic barley lines with up to 94.7 % reduction in the levels of C-hordein protein relative to the parental line. The composition of the prolamin fraction of the barley parental line cv. Golden Promise was resolved using SDS...

Induced resistance by cresotic acid (3-hydroxy-4-methyl methylbenzoic acid) against wilt disease of melon and cotton

International Nuclear Information System (INIS)

Dong, H.; Li, Z.; Zhang, D.; Li, W.; Tang, W.

2004-01-01

Cresotic acid (3-hydroxy-4-methylbenzoic acid) was proved be active in controlling wilt diseases of melon and cotton plants grown in the house. Soil drench with 200-1000 ppm cresotic acid induced 62-77 %, 69-79 % and 50-60 % protection against Fusarium oxysporum f.sp melonis (FOM) in melon, Fusarium oxysporum f.sp vasinfectum (FOV) and Verticillium dahliae in cotton, respectively. Since no inhibitory effect of cresotic acid on mycelial growth of these three fungual pathogens was observed in vitro, it is suggested that control of these wilt diseases with cresotic acid resulted from induced resistance. Cresotic acid induced resistance in melon plants not only against race 0, race 1, race 2 and race 1,2, but also against a mixture of these four races of FOM, suggesting a non-race- specific resistance. Level of induced resistance by cresotic acid against FOM depended on inoculum pressure applied to melon plants. At 25 day after inoculation with FOM, percentage protection induced by cresotic acid under low inoculum pressure retained a level of 51 %, while under high inoculum pressure percentage protection decreased to only 10 %. High concentrations of cresotic acid significantly reduced plant growth. Reduction in fresh weight of melon (36-51%) and cotton (42-71%) was obtained with 500-1000 ppm cresotic acid, while only less than 8% reduction occurred with 100-200 ppm. (author)

Application of 5-Aminosalicylic Acid Preparations in the Treatment of Inflammatory Bowel Diseases

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Yu.M. Stepanov

2016-09-01

Full Text Available The article is devoted to the comparative characterization of different derivatives of 5-aminosalicylic acid. Researches of the past decades have changed the presentation of the potential use of aminosalicylates in the therapy of various inflammatory bowel diseases. In connection with unknown etiology of Crohn’s disease and ulcerative colitis, there is no causal treatment of these diseases. The essence of treatment is reduced to inhibition of inflammatory activity during exacerbations and a course of preventive treatment. Numerous studies over the past 20 years have shown that the basis of basic treatment for nonspecific chronic inflammatory bowel diseases is 5-aminosalicylic acid drugs, or salicylates. When choosing the dosage form, you should take into account the differences between mesalazine preparations depending on the type of the coat. It is shown that in terms of pharmacokinetics, the most effective mesalazine dosage forms for the treatment of ulcerative colitis and Crohn’s disease with lesions of the colon are enteric coated tablets that provides a pH-dependent gradual release of 5-aminosalicylic acid throughout the entire colon. 5-aminosalicylic acid drugs available today on the pharmaceutical market are able to control the course of ulcerative colitis and Crohn’s disease with lesions of the colon in the majority of patients. The use of 5-aminosalicylic acid preparations is not limited to the treatment of ulcerative colitis and Crohn’s disease. The drug is widely used in other diseases of the bowel, such as diverticular disease of the colon, colitis banal, radiation damages of the colon, as well as to treat common diseases, such as irritable bowel syndrome. The study, which was conducted in the department bowel disease of the State Institution «Institute of Gastroenterology» for 2 years, showed a positive effect of 20-day treatment with Mesacol in uncomplicated diverticular disease. The use of Mesacol at a dose of 1,600 mg

Temperature Affects the Use of Storage Fatty Acids as Energy Source in a Benthic Copepod (Platychelipus littoralis, Harpacticoida).

Science.gov (United States)

Werbrouck, Eva; Van Gansbeke, Dirk; Vanreusel, Ann; De Troch, Marleen

2016-01-01

The utilization of storage lipids and their associated fatty acids (FA) is an important means for organisms to cope with periods of food shortage, however, little is known about the dynamics and FA mobilization in benthic copepods (order Harpacticoida). Furthermore, lipid depletion and FA mobilization may depend on the ambient temperature. Therefore, we subjected the temperate copepod Platychelipus littoralis to several intervals (3, 6 and 14 days) of food deprivation, under two temperatures in the range of the normal habitat temperature (4, 15 °C) and under an elevated temperature (24 °C), and studied the changes in FA composition of storage and membrane lipids. Although bulk depletion of storage FA occurred after a few days of food deprivation under 4 °C and 15 °C, copepod survival remained high during the experiment, suggesting the catabolization of other energy sources. Ambient temperature affected both the degree of FA depletion and the FA mobilization. In particular, storage FA were more exhausted and FA mobilization was more selective under 15 °C compared with 4 °C. In contrast, depletion of storage FA was limited under an elevated temperature, potentially due to a switch to partial anaerobiosis. Food deprivation induced selective DHA retention in the copepod's membrane, under all temperatures. However, prolonged exposure to heat and nutritional stress eventually depleted DHA in the membranes, and potentially induced high copepod mortality. Storage lipids clearly played an important role in the short-term response of the copepod P. littoralis to food deprivation. However, under elevated temperature, the use of storage FA as an energy source is compromised.

Prolonged maintenance of 2,3-diphosphoglycerate acid and adenosine triphosphate in red blood cells during storage.

Science.gov (United States)

de Korte, Dirk; Kleine, Mya; Korsten, Herbert G H; Verhoeven, Arthur J

2008-06-01

Current additive solutions (ASs) for red cells (RBCs) do not maintain a constant level of critical metabolites such as adenosine triphosphate (ATP) and 2,3-diphosphoglycerate acid (2,3-DPG) during cold storage. From the literature it is known that the intracellular pH is an important determinant of RBC metabolism. Therefore, a new, alkaline, AS was developed with the aim to allow cold storage of RBCs with stable product characteristics. Whole blood-derived RBCs (leukoreduced) were resuspended in experimental medium phosphate-adenine-guanosine-glucose-gluconate-mannitol (PAGGG-M; pH 8.2) with and without washing in the same medium. During cold storage several in vitro variables, such as intracellular pH, 2,3-DPG, ATP, and hemolysis, were analyzed. During cold storage, RBCs resuspended in PAGGG-M showed a constant ATP level (approx. 6 mumol/g Hb) and a very limited hemolysis (level), followed by a slow decrease, with at Day 35 still 100 percent of the initial level. RBCs washed in PAGGG-M even showed a continuous increase of 2,3-DPG during 35 days, with a maximum level of 200 percent of the initial value. The effect of PAGGG-M appears to be related to long-lasting effects of the initial intracellular pH shortly after production. Resuspension of RBCs in our alkaline medium PAGGG-M resulted in a RBC unit of high quality during storage for up to at least 35 days, with 2,3-DPG levels of higher than 10 mumol per g Hb, hemolysis of less than 0.2 percent, and ATP levels of higher than 5 mumol per g Hb.

Effects of bile acids on human airway epithelial cells: implications for aerodigestive diseases

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Adil Aldhahrani

2017-03-01

Full Text Available Gastro-oesophageal reflux and aspiration have been associated with chronic and end-stage lung disease and with allograft injury following lung transplantation. This raises the possibility that bile acids may cause lung injury by damaging airway epithelium. The aim of this study was to investigate the effect of bile acid challenge using the immortalised human bronchial epithelial cell line (BEAS-2B. The immortalised human bronchial epithelial cell line (BEAS-2B was cultured. A 48-h challenge evaluated the effect of individual primary and secondary bile acids. Post-challenge concentrations of interleukin (IL-8, IL-6 and granulocyte−macrophage colony-stimulating factor were measured using commercial ELISA kits. The viability of the BEAS-2B cells was measured using CellTiter-Blue and MTT assays. Lithocholic acid, deoxycholic acid, chenodeoxycholic acid and cholic acid were successfully used to stimulate cultured BEAS-2B cells at different concentrations. A concentration of lithocholic acid above 10 μmol·L−1 causes cell death, whereas deoxycholic acid, chenodeoxycholic acid and cholic acid above 30 μmol·L−1 was required for cell death. Challenge with bile acids at physiological levels also led to a significant increase in the release of IL-8 and IL6 from BEAS-2B. Aspiration of bile acids could potentially cause cell damage, cell death and inflammation in vivo. This is relevant to an integrated gastrointestinal and lung physiological paradigm of chronic lung disease, where reflux and aspiration are described in both chronic lung diseases and allograft injury.

Studies on the storage of irradiated potatoes

Energy Technology Data Exchange (ETDEWEB)

Sharabash, M; Orabi, I O [National Center for research and radiation Technology, Nasr City, Cairo (Egypt); Eloksh, I I; Abd-Alia, M A [Faculty of Agric. Ain Shams University, Cairo (Egypt)

1995-10-01

Tubers of alpha and king edward potato cultivars were exposed to O, 10 or 50 krad and stored under room temperature and good ventilation in perforated standard carton boxes. 10 Krad was the promising dosage for sprouting inhibition for both the two cultivars. Total losses in weight were partially dependent on the volume of the dosage, the time elapsed after irradiation and cultivar used. Ascorbic acid slightly decreased, whilst sugar fractions were increased during storage in irradiated tubers. Sugar fractions were sharply decreased in boiled or fried potatoes. Also, chlorogenic acid was increased by prolonging the storage time and/or increasing the exposure dose. After-cooking discoloration (darkening) was reduced by gamma irradiation and/or extending the storage period. Using 0.5% citric acid or 0.5% sodium citrate solution inhibited the after-cooking discoloration. 8 tabs.

Studies on the storage of irradiated potatoes

International Nuclear Information System (INIS)

Sharabash, M.; Orabi, I.O.; Eloksh, I.I.; Abd-Alia, M.A.

1995-01-01

Tubers of alpha and king edward potato cultivars were exposed to O, 10 or 50 krad and stored under room temperature and good ventilation in perforated standard carton boxes. 10 Krad was the promising dosage for sprouting inhibition for both the two cultivars. Total losses in weight were partially dependent on the volume of the dosage, the time elapsed after irradiation and cultivar used. Ascorbic acid slightly decreased, whilst sugar fractions were increased during storage in irradiated tubers. Sugar fractions were sharply decreased in boiled or fried potatoes. Also, chlorogenic acid was increased by prolonging the storage time and/or increasing the exposure dose. After-cooking discoloration (darkening) was reduced by gamma irradiation and/or extending the storage period. Using 0.5% citric acid or 0.5% sodium citrate solution inhibited the after-cooking discoloration. 8 tabs

Omega-3 Fatty Acids in Early Prevention of Inflammatory Neurodegenerative Disease: A Focus on Alzheimer’s Disease

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J. Thomas

2015-01-01

Full Text Available Alzheimer’s disease (AD is the leading cause of dementia and the most common neurodegenerative disease in the elderly. Furthermore, AD has provided the most positive indication to support the fact that inflammation contributes to neurodegenerative disease. The exact etiology of AD is unknown, but environmental and genetic factors are thought to contribute, such as advancing age, family history, presence of chronic diseases such as cardiovascular disease (CVD and diabetes, and poor diet and lifestyle. It is hypothesised that early prevention or management of inflammation could delay the onset or reduce the symptoms of AD. Normal physiological changes to the brain with ageing include depletion of long chain omega-3 fatty acids and brains of AD patients have lower docosahexaenoic acid (DHA levels. DHA supplementation can reduce markers of inflammation. This review specifically focusses on the evidence in humans from epidemiological, dietary intervention, and supplementation studies, which supports the role of long chain omega-3 fatty acids in the prevention or delay of cognitive decline in AD in its early stages. Longer term trials with long chain omega-3 supplementation in early stage AD are warranted. We also highlight the importance of overall quality and composition of the diet to protect against AD and dementia.

Acid sphingomyelinase (Asm) deficiency patients in The Netherlands and Belgium: disease spectrum and natural course in attenuated patients.

Science.gov (United States)

Hollak, C E M; de Sonnaville, E S V; Cassiman, D; Linthorst, G E; Groener, J E; Morava, E; Wevers, R A; Mannens, M; Aerts, J M F G; Meersseman, W; Akkerman, E; Niezen-Koning, K E; Mulder, M F; Visser, G; Wijburg, F A; Lefeber, D; Poorthuis, B J H M

2012-11-01

Niemann-Pick disease (NPD) is a neurovisceral lysosomal storage disorder caused by acid sphingomyelinase (ASM) deficiency, which can be categorized as either Niemann-Pick disease type A [NPD-A], with progressive neurological disease and death in early childhood, or as Niemann-Pick disease type B [NPD-B], with a more variable spectrum of manifestations. Enzyme replacement therapy (ERT) with recombinant sphingomyelinase is currently studied as potential treatment for NPD-B patients. The objective of this study is to characterize the clinical features of patients with ASM deficiency in the Netherlands and Belgium with focus on the natural disease course of NPD-B patients. Prospective and retrospective data on ASM deficient patients were collected in The Netherlands and part of Belgium. Patients with NPD-B that could be followed prospectively were evaluated every 6-12 months for pulmonary function tests, 6 minute walk test (6 MWT), imaging (bone marrow infiltration measured by QCSI, organ volumes by MRI and CT scan of the lungs) and biochemical markers. Twenty-five patients with ASM deficiency were identified (13 males, 12 females, median age 13years, range 1-59 years). Nine patients had died at the time of the study, including four NPD-A patients at the age of 1,1, 2, 3 and five NPDB patents at the age of 5, 6, 43, 56 and 60 years. There was a high prevalence of homozygosity and compound heterozygosity for the common p.Arg608del mutation in 43% and 19% of NPD-B patients, respectively. In NPD-B patients, thrombocytopenia was present in most, while anemia and leucopenia were less common (33% and 6 % respectively). HDL cholesterol was reduced in most patients. Pulmonary disease was severe in several patients. Follow-up up to 11 years revealed a gradual decrease in platelet count. Detailed investigations in 6 NPD-B patients with follow-up in 4 patients revealed remarkable stable disease parameters up to 6 years, with some decline in pulmonary function and 6 MWT. Bone

Prevention of Lysosomal Storage Diseases and Derivation of Mutant Stem Cell Lines by Preimplantation Genetic Diagnosis

Science.gov (United States)

Altarescu, Gheona; Beeri, Rachel; Eiges, Rachel; Epsztejn-Litman, Silvina; Eldar-Geva, Talia; Elstein, Deborah; Zimran, Ari; Margalioth, Ehud J.; Levy-Lahad, Ephrat; Renbaum, Paul

2012-01-01

Preimplantation genetic diagnosis (PGD) allows birth of unaffected children for couples at risk for a genetic disorder. We present the strategy and outcome of PGD for four lysosomal storage disorders (LSD): Tay-Sachs disease (TSD), Gaucher disease (GD), Fabry disease (FD), and Hunter syndrome (HS), and subsequent development of stem cell lines. For each disease, we developed a family-specific fluorescent multiplex single-cell PCR protocol that included the familial mutation and informative markers surrounding the mutation. Embryo biopsy and PGD analysis were performed on either oocytes (polar bodies one and two) or on single blastomeres from a six-cell embryo. We treated twenty families carrying mutations in these lysosomal storage disorders, including 3 couples requiring simultaneous analysis for two disorders (TSD/GD, TSD/balanced Robertsonian translocation 45XYder(21;14), and HS/oculocutaneus albinism). These analyses led to an overall pregnancy rate/embryo transfer of 38% and the birth of 20 unaffected children from 17 families. We have found that PGD for lysosomal disorders is a safe and effective method to prevent birth of affected children. In addition, by using mutant embryos for the derivation of stem cell lines, we have successfully established GD and HS hESC lines for use as valuable models in LSD research. PMID:23320174

Pre-harvest application of salicylic acid maintain the rind textural properties and reduce fruit rot and chilling injury of sweet orange during cold storage

International Nuclear Information System (INIS)

Ahmad, S.; Khan, A.D.; Iqbal, Z.; Singh, Z.; Iqbal, Z.

2013-01-01

Trees of citrus cvs Lane Late and Valencia Late oranges were sprayed ten days before anticipated harvest with salicylic acid (SA) at different concentrations (2, 3, 4, 6, 8, 9 mM). Fruits were harvested and stored at 5 degree C for 93 days to investigate the effects of SA on fruit rot, chilling injury and quality relating parameters such as rind and fruit firmness as well as sugar and organic acid contents. Fruits were analyzed before storage and after 31, 62 and 93 days of storage and found that SA at 8 mM and 9 mM reduced fruit rot from 16.93% to 6.06% and 12.78% to 5.12% in Lane Late and Valencia Late, respectively. Chilling injury was significantly reduced at 8 mM and 9 mM treatments. Textural properties relating to rind puncture, rind tensile and fruit firmness forces showed that treated fruits were significantly firmer than those of control. Maintained contents of SSC, TA, individual sugars and organic acids in treated fruit with higher doses after 93 confirmed preliminary findings such as SA has anti-senescent effect. Our research suggests that pre-harvest spray of SA can be used effectively to minimize the post-harvest/storage losses of sweet oranges cultivars. (author)

Gut microbiota–derived short-chain fatty acids and kidney diseases

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Li L

2017-12-01

Full Text Available Lingzhi Li, Liang Ma, Ping Fu Kidney Research Institute, Department of Nephrology, West China Hospital of Sichuan University, Chengdu 610041, China Abstract: Gut microbiota and its metabolites play pivotal roles in host physiology and pathology. Short-chain fatty acids (SCFAs, as a group of metabolites, exert positive regulatory effects on energy metabolism, hormone secretion, immune inflammation, hypertension, and cancer. The functions of SCFAs are related to their activation of transmembrane G protein-coupled receptors and their inhibition of histone acetylation. Though controversial, growing evidence suggests that SCFAs, which regulate inflammation, oxidative stress, and fibrosis, have been involved in kidney disease through the activation of the gut–kidney axis; however, the molecular relationship among gut microbiota–derived metabolites, signaling pathways, and kidney disease remains to be elucidated. This review will provide an overview of the physiology and functions of SCFAs in kidney disease. Keywords: gut microbiome, short-chain fatty acids, kidney diseases, gut–kidney axis

Electroactive and High Dielectric Folic Acid/PVDF Composite Film Rooted Simplistic Organic Photovoltaic Self-Charging Energy Storage Cell with Superior Energy Density and Storage Capability.

Science.gov (United States)

Roy, Swagata; Thakur, Pradip; Hoque, Nur Amin; Bagchi, Biswajoy; Sepay, Nayim; Khatun, Farha; Kool, Arpan; Das, Sukhen

2017-07-19

Herein we report a simplistic prototype approach to develop an organic photovoltaic self-charging energy storage cell (OPSESC) rooted with biopolymer folic acid (FA) modified high dielectric and electroactive β crystal enriched poly(vinylidene fluoride) (PVDF) composite (PFA) thin film. Comprehensive and exhaustive characterizations of the synthesized PFA composite films validate the proper formation of β-polymorphs in PVDF. Significant improvements of both β-phase crystallization (F(β) ≈ 71.4%) and dielectric constant (ε ≈ 218 at 20 Hz for PFA of 7.5 mass %) are the twosome realizations of our current study. Enhancement of β-phase nucleation in the composites can be thought as a contribution of the strong interaction of the FA particles with the PVDF chains. Maxwell-Wagner-Sillars (MWS) interfacial polarization approves the establishment of thermally stable high dielectric values measured over a wide temperature spectrum. The optimized high dielectric and electroactive films are further employed as an active energy storage material in designing our device named as OPSESC. Self-charging under visible light irradiation without an external biasing electrical field and simultaneous remarkable self-storage of photogenerated electrical energy are the two foremost aptitudes and the spotlight of our present investigation. Our as fabricated device delivers an impressively high energy density of 7.84 mWh/g and an excellent specific capacitance of 61 F/g which is superior relative to the other photon induced two electrode organic self-charging energy storage devices reported so far. Our device also proves the realistic utility with good recycling capability by facilitating commercially available light emitting diode.

Roles of amino acids in preventing and treating intestinal diseases: recent studies with pig models.

Science.gov (United States)

Liu, Yulan; Wang, Xiuying; Hou, Yongqing; Yin, Yulong; Qiu, Yinsheng; Wu, Guoyao; Hu, Chien-An Andy

2017-08-01

Animal models are needed to study and understand a human complex disease. Because of their similarities in anatomy, structure, physiology, and pathophysiology, the pig has proven its usefulness in studying human gastrointestinal diseases, such as inflammatory bowel disease, ischemia/reperfusion injury, diarrhea, and cancer. To understand the pathogenesis of these diseases, a number of experimental models generated in pigs are available, for example, through surgical manipulation, chemical induction, microbial infection, and genetic engineering. Our interests have been using amino acids as therapeutics in pig and human disease models. Amino acids not only play an important role in protein biosynthesis, but also exert significant physiological effects in regulating immunity, anti-oxidation, redox regulation, energy metabolism, signal transduction, and animal behavior. Recent studies in pigs have shown that specific dietary amino acids can improve intestinal integrity and function under normal and pathological conditions that protect the host from different diseases. In this review, we summarize several pig models in intestinal diseases and how amino acids can be used as therapeutics in treating pig and human diseases.

Outcomes in patients with nonerosive reflux disease treated with a proton pump inhibitor and alginic acid ± glycyrrhetinic acid and anthocyanosides

Directory of Open Access Journals (Sweden)

Di Pierro F

2013-03-01

Full Text Available Francesco Di Pierro,1 Mario Gatti,2 Giuliana Rapacioli,3 Leandro Ivaldi4 1Velleja Research, Milan, 2Gastroenterology Department, Giussano Hospital, Monza-Brianza, 3AIOR, Piacenza, 4Digestive Endoscopic Department, Ceva Hospital, Ceva, Cuneo, Italy Background: The purpose of this study was to compare the efficacy of alginic acid alone versus alginic acid combined with low doses of pure glycyrrhetinic acid and bilberry anthocyanosides as an addon to conventional proton pump inhibitor therapy in relieving symptoms associated with nonerosive reflux disease. Methods: This prospective, randomized, 8-week, open-label trial was conducted at two centers. Sixty-three patients with persistent symptoms of gastroesophageal reflux disease and normal upper gastrointestinal endoscopy were eligible for the study. Patients in group A (n = 31 were treated with pantoprazole and a formula (Mirgeal® containing alginic acid and low doses of pure glycyrrhetinic acid + standardized Vaccinium myrtillus extract for 4 weeks, then crossed over to the multi-ingredient formula for a further 4 weeks. Patients in group B (n = 32 were treated pantoprazole and alginic acid alone twice daily, then crossed over to alginic acid twice daily for a further 4 weeks. Efficacy was assessed by medical evaluation of a symptom relief score, estimated using a visual analog scale (0–10. Side effects, tolerability, and compliance were also assessed. Results: Of the 63 patients enrolled in the study, 58 (29 in group A and 29 in group B completed the 8-week trial. The baseline characteristics were comparable between the two groups. During the study, significant differences were recorded in symptom scores for both groups. In group A, symptoms of chest pain, heartburn, and abdominal swelling were less serious than in group B. Treatment A was better tolerated, did not induce hypertension, and had fewer side effects than treatment B. No significant differences in compliance were found between the

Probiotic viability and storage stability of yogurts and fermented milks prepared with several mixtures of lactic acid bacteria.

Science.gov (United States)

Mani-López, E; Palou, E; López-Malo, A

2014-05-01

Currently, the food industry wants to expand the range of probiotic yogurts but each probiotic bacteria offers different and specific health benefits. Little information exists on the influence of probiotic strains on physicochemical properties and sensory characteristics of yogurts and fermented milks. Six probiotic yogurts or fermented milks and 1 control yogurt were prepared, and we evaluated several physicochemical properties (pH, titratable acidity, texture, color, and syneresis), microbial viability of starter cultures (Lactobacillus delbrueckii ssp. bulgaricus and Streptococcus thermophilus) and probiotics (Lactobacillus acidophilus, Lactobacillus casei, and Lactobacillus reuteri) during fermentation and storage (35 d at 5°C), as well as sensory preference among them. Decreases in pH (0.17 to 0.50 units) and increases in titratable acidity (0.09 to 0.29%) were observed during storage. Only the yogurt with S. thermophilus, L. delbrueckii ssp. bulgaricus, and L. reuteri differed in firmness. No differences in adhesiveness were determined among the tested yogurts, fermented milks, and the control. Syneresis was in the range of 45 to 58%. No changes in color during storage were observed and no color differences were detected among the evaluated fermented milk products. Counts of S. thermophilus decreased from 1.8 to 3.5 log during storage. Counts of L. delbrueckii ssp. bulgaricus also decreased in probiotic yogurts and varied from 30 to 50% of initial population. Probiotic bacteria also lost viability throughout storage, although the 3 probiotic fermented milks maintained counts ≥ 10(7)cfu/mL for 3 wk. Probiotic bacteria had variable viability in yogurts, maintaining counts of L. acidophilus ≥ 10(7) cfu/mL for 35 d, of L. casei for 7d, and of L. reuteri for 14 d. We found no significant sensory preference among the 6 probiotic yogurts and fermented milks or the control. However, the yogurt and fermented milk made with L. casei were better accepted. This

Stability of the pyrethroid pesticide bifenthrin in milled wheat during thermal processing, yeast and lactic acid fermentation, and storage.

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Dorđević, Tijana M; Šiler-Marinković, Slavica S; Ðurović, Rada D; Dimitrijević-Branković, Suzana I; Gajić Umiljendić, Jelena S

2013-10-01

Pesticide residues have become an unavoidable part of food commodities. In the context of increased interest for food processing techniques as a tool for reducing pesticide residues, it is interesting to study the potential loss of pesticides during lactic acid and yeast fermentation. In the present paper the effect of fermentation by Lactobacillus plantarum and Saccharomyces cerevisiae and storage on 23 °C on bifenthrin in wheat was investigated. In addition, the effect of sterilisation (applied in order to avoid contamination with wild microorganism strains, i.e. to determine the individual effects of used strains) on bifenthrin degradation was tested as well. No significant loss of bifenthrin was observed during storage, or after the sterilisation. During the lactic acid fermentation, reduction within wheat fortified with 0.5 mg kg(-1) was 42%, while quite lower within samples fortified with 2.5 mg kg(-1) , maximum 18%. In contrast, bifenthrin concentration was not reduced during yeast fermentation, as the reduction in fortified samples was in the range of spontaneous chemical degradation during incubation period. Possible bifenthrin contamination in wheat, in amounts over the maximum residue limits, could not be reduced by sterilisation or by yeast fermentation, but lactic acid fermentation could be an effective tool for minimising residual contamination. © 2013 Society of Chemical Industry.

Aristolochic acid nephropathy: Harbinger of a global iatrogenic disease.

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Grollman, Arthur P

2013-01-01

This review constitutes an overview of our investigations of aristolochic acid nephropathy, a chronic kidney disease associated with carcinomas of the upper urinary tract. Our studies began by confirming the hypothesis that chronic dietary poisoning by aristolochic acid was responsible for endemic (Balkan) nephropathy. A unique TP53 mutational signature in urothelial tumors and the presence of aristolactam-DNA adducts in the renal cortex, defined in the course of this research, proved to be robust biomarkers of exposure to this potent nephrotoxin and human carcinogen. Armed with this information, we used molecular epidemiologic approaches and novel mechanistic information to establish the causative role of aristolochic acid in upper urinary tract carcinoma in Taiwan, where one-third of the population had been prescribed herbal remedies containing Aristolochia, and the recorded incidence of upper urinary tract cancers is the highest in the world. As traditional Chinese medicine is practiced similarly in Taiwan and China, it is likely that upper urinary tract carcinomas and their attendant aristolochic acid nephropathy are prevalent in China and other Asian countries where Aristolochia herbs have been used for centuries in the treatment and prevention of disease, creating a potential public health problem of considerable magnitude. Copyright © 2012 Wiley Periodicals, Inc.

The effects of pre-harvest napthalene acetic acid and aminoethoxyvinylglycine treatments on storage performance of ‘ Ak Sakı’ apple cultivar grown in Erzincan conditions

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Burhan OZTÜRK

2013-06-01

Full Text Available This study was carried out to determine the effects of pre-harvest aminoethoxyvinylglycine (AVG, 150, 225 ve 300 mg/L and naphthaleneacetic acid (NAA, 20 mg/L treatments in different doses on storage performance of ‘Ak Sakı’ apple cultivar (Malus domestica Borkh. in 2012. The changes on some fruit quality parameters were measured at 2±1 oC temperature and with 90±5 % relative humidity at 45 days interval during storage. The lowest weight loss was obtained from 300 mg/L AVG treated fruits during the storage. In the all analysis date, the highest L* value was obtained from 300 mg/L AVG treated fruits, and the lowest hue angle value was reported from the fruits of control treatment. The flesh firmness was determined that the best kept in the 225 and 300 mg/L AVG treated fruits during the storage. The flesh firmness significantly reduced with NAA treatment at the end of storage. The highest soluble solids concentration (SSC was obtain from control fruit during the storage, whereas the lowest SSC was observed in fruit treated with 300 mg/L AVG. In the all analysis date, the highest titratable acidity was obtained in fruits treated with 225 and 300 mg/L AVG. The starch degradation was delayed with AVG treatments.

Impaired Autophagy in the Lipid-Storage Disorder Niemann-Pick Type C1 Disease

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Sovan Sarkar

2013-12-01

Full Text Available Autophagy dysfunction has been implicated in misfolded protein accumulation and cellular toxicity in several diseases. Whether alterations in autophagy also contribute to the pathology of lipid-storage disorders is not clear. Here, we show defective autophagy in Niemann-Pick type C1 (NPC1 disease associated with cholesterol accumulation, where the maturation of autophagosomes is impaired because of defective amphisome formation caused by failure in SNARE machinery, whereas the lysosomal proteolytic function remains unaffected. Expression of functional NPC1 protein rescues this defect. Inhibition of autophagy also causes cholesterol accumulation. Compromised autophagy was seen in disease-affected organs of Npc1 mutant mice. Of potential therapeutic relevance is that HP-β-cyclodextrin, which is used for cholesterol-depletion treatment, impedes autophagy, whereas stimulating autophagy restores its function independent of amphisome formation. Our data suggest that a low dose of HP-β-cyclodextrin that does not perturb autophagy, coupled with an autophagy inducer, may provide a rational treatment strategy for NPC1 disease.

Metabolomic approach: postharvest storage stability of red radish (raphanus sativus l.)

International Nuclear Information System (INIS)

Jahangir, M.; Farid, J.B.A.

2014-01-01

Post harvest storage of vegetables at different temperature for consumption is commonly practiced that need standardization. Among vegetables, red radish (Raphanus sativus L.) is a well known and commonly consumed vegetable all over the world. Its bioactive or nutritional constituents include a wide range of metabolites including, glucosinolates, phenolics, amino acids, organic acids, and sugars. However, many of these metabolites are not stable and can easily be degraded or modified during storage. In order to investigate the metabolomic changes during post harvest storage, radish samples (intact roots and aerial parts) were subjected to four different storage temperatures above and below 0 degree C (20 degree C, 4 degree C, -20 degree C, and -80 degree C), for a maximum of 28 days. 1H-NMR and two-dimensional NMR spectra data resulting from the analysis of the different samples were subjected to principal component analysis (PCA) to investigate any possible metabolomic changes. A profound chemical alteration was observed in primary and secondary metabolites. Glucosinolates, phenylpropanoids, organic acids, amino acids, and sugars were found to be the discriminating metabolites for the storage effect. Initially, an increase in secondary metabolites (phenolics and glucosinolates) was observed, but levels of these compounds decreased in later stages, probably due to the breakdown of these products. Whereas late storage samples contained high amounts of amino acids (alanine, valine, threonine, (gama-amino-butyric acid / GABA)) and some glucosinolates (glucobrassicin, neoglucobrassicin). This phenomenon was pronounced at room temperature as compared to other storage temperatures. Interestingly even at lower and freezing temperatures metabolomic changes in these biological samples were observed. The least metabolomic changes were observed at samples stored at -80 degree C. While studying temperature dependent metabolomic changes, high levels of glucose, adenine, alanine

Concentrations of free amino acids and sugars in nine potato varieties: effects of storage and relationship with acrylamide formation.

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Halford, Nigel G; Muttucumaru, Nira; Powers, Stephen J; Gillatt, Peter N; Hartley, Lee; Elmore, J Stephen; Mottram, Donald S

2012-12-05

Acrylamide forms during cooking and processing predominately from the reaction of free asparagine and reducing sugars in the Maillard reaction. The identification of low free asparagine and reducing sugar varieties of crops is therefore an important target. In this study, nine varieties of potato (French fry varieties Maris Piper (from two suppliers), Pentland Dell, King Edward, Daisy, and Markies; and chipping varieties Lady Claire, Lady Rosetta, Saturna, and Hermes) grown in the United Kingdom in 2009 were analyzed at monthly intervals through storage from November 2009 to July 2010. Acrylamide formation was measured in heated flour and chips fried in oil. Analysis of variance revealed significant interactions between varieties nested within type (French fry and chipping) and storage time for most free amino acids, glucose, fructose, and acrylamide formation. Acrylamide formed in chips correlated significantly with acrylamide formed in flour and with chip color. There were significant correlations between glucose or total reducing sugar concentration and acrylamide formation in both variety types, but with fructose the correlation was much stronger for chipping than for French fry varieties. Conversely, there were significant correlations with acrylamide formation for both total free amino acid and free asparagine concentration in the French fry but not chipping varieties. The study showed the potential of variety selection for preventing unacceptable levels of acrylamide formation in potato products and the variety-dependent effect of long-term storage on acrylamide risk. It also highlighted the complex relationship between precursor concentration and acrylamide risk in potatoes.

Viability of lactic acid bacteria coated as synbiotic during storage and gastro-intestinal simulation

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Jamilah, It; Priyani, Nunuk; Lusia Natalia, Santa

2018-03-01

Lactic acid bacteria (LAB) has been added to various food products as a probiotic agent because it has been known to provide beneficial health effects in humans. In the application of LAB, cell viability often decreased as influenced by environment stresses. Encapsulation technique is one of the cell protection techniques using a coating material. Effective coating material is required to produce maximum protection of LAB cells. In this study, candidate of probiotic LAB (isolate US7) was encapsulated with alginate-mung bean flour and alginate-gram flour with inulin prebiotic by extrusion technique. Viability of encapsulated LAB cells were able to survive by up to 108CFU g‑1 after 4 weeks of storage at 4 °C. Beads were incubated in simulated liquid gastric acid (pH=2) for 2 hrs and simulated intestinal fluid (pH=6) for 3 hrs at 37 °C. The results showed that encapsulated LAB cells maintained the survival rate of 97% with the number of cells at 9.07 Log CFU g‑1in the simulated liquid gastric acid and then followed by releasing cells in simulated intestinal fluid. In general, this study indicates that encapsulation with alginate-mung bean flour and alginategram flour with inulin successfullyprotect probiotic bacteria against simulated human gastrointestinal conditions.

Enrichment of Cinta Senese burgers with omega-3 fatty acids. Effect of type of addition and storage conditions on quality characteristics

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C. Aquilani

2018-03-01

Full Text Available The most beneficial omega-3 PUFAs to human health, EPA and DHA fatty acids, are typically present in fish products, but extraneous to meat. Therefore, Cinta Senese pork burgers were added with microencapsulated (M and bulk fish oil (F and subjected to three storage conditions: no storage (T0, chilled (T5 and frozen storage (T30. The physico-chemical and sensory attributes of raw and cooked burgers were investigated. After storage and cooking, EPA and DHA were better preserved in M burgers than in F samples, which showed the highest TBAR values at T0 and T5, while M samples presented scores similar to the control. Panelists observed differences mainly in greasy appearance, odor intensity and cooked meat odor and flavor. The M group showed the best scores at T5 with respect to the control and F burgers. So, fish oil microencapsulation was an effective method to prevent EPA and DHA oxidation while respecting burger quality characteristics.

Iron storage disease (hemochromatosis) and hepcidin response to iron load in two species of pteropodid fruit bats relative to the common vampire bat.

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Stasiak, Iga M; Smith, Dale A; Ganz, Tomas; Crawshaw, Graham J; Hammermueller, Jutta D; Bienzle, Dorothee; Lillie, Brandon N

2018-07-01

Hepcidin is the key regulator of iron homeostasis in the body. Iron storage disease (hemochromatosis) is a frequent cause of liver disease and mortality in captive Egyptian fruit bats (Rousettus aegyptiacus), but reasons underlying this condition are unknown. Hereditary hemochromatosis in humans is due to deficiency of hepcidin or resistance to the action of hepcidin. Here, we investigated the role of hepcidin in iron metabolism in one species of pteropodid bat that is prone to iron storage disease [Egyptian fruit bat (with and without hemochromatosis)], one species of pteropodid bat where iron storage disease is rare [straw-colored fruit bat (Eidolon helvum)], and one species of bat with a natural diet very high in iron, in which iron storage disease is not reported [common vampire bat (Desmodus rotundus)]. Iron challenge via intramuscular injection of iron dextran resulted in significantly increased liver iron content and histologic iron scores in all three species, and increased plasma iron in Egyptian fruit bats and straw-colored fruit bats. Hepcidin mRNA expression increased in response to iron administration in healthy Egyptian fruit bats and common vampire bats, but not in straw-colored fruit bats or Egyptian fruit bats with hemochromatosis. Hepcidin gene expression significantly correlated with liver iron content in Egyptian fruit bats and common vampire bats, and with transferrin saturation and plasma ferritin concentration in Egyptian fruit bats. Induction of hepcidin gene expression in response to iron challenge is absent in straw-colored fruit bats and in Egyptian fruit bats with hemochromatosis and, relative to common vampire bats and healthy humans, is low in Egyptain fruit bats without hemochromatosis. Limited hepcidin response to iron challenge may contribute to the increased susceptibility of Egyptian fruit bats to iron storage disease.

Wol man's disease: The King Faisal Specialist Hospital and Research Centre Experience

International Nuclear Information System (INIS)

Al-Essa, M.; Sakati, N.; Joshi, S.; Ozand, P.T.; Nounou, R.; Le Quesne, G.; Archibald, A.

1998-01-01

Wolman's disease is a rare autosomal recessive lysosomal storage disease. A recent review indicates that approximately 50 patients have reported in the world. Reports of patients from the Arabian Peninsula are rare due to lack of awareness among pediatricians. We retrospectively reviewed the clinical, radiological, biochemical and histopathological findings of four Saudi patients diagnosed with Wolman's disease at King Faisal Hospital and Research Centre. The diagnosis was confirmed by deficient acid lipase activity in the leukocytes and fibroblasts, which was measured using 4-methylumbelliferyl palmitate. All patients were failing to thrive with progressive heptasplenomegaly. Abdominal x-ray revealed calcifications which were confirmed on abdominal CT scan. Peripheral blood film showed foamy histiocytes. Liver biopsy in one patient showed marked steatosis and elliptical empty clefs predominantly in the Kupffer cells, indicating cholesterol storage in the reticulo-endothelial cells. The acid lipase activity was less than 6% in all patients. In all suspected cases of Wolman's disease, a plane abdominal x-ray should be obtained to check for the typical pattern of adrenal calcification characteristic of the disease, especially ant young infant with failure to thrive and progressive hepatosplenomegaly. (author)

Adrenic acid as an inflammation enhancer in non-alcoholic fatty liver disease.

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Horas H Nababan, Saut; Nishiumi, Shin; Kawano, Yuki; Kobayashi, Takashi; Yoshida, Masaru; Azuma, Takeshi

2017-06-01

This study was designed to identify novel links between lipid species and disease progression in non-alcoholic fatty liver disease (NAFLD). We analyzed lipid species in the liver and plasma of db/db mice fed a choline-deficient l-amino acid-defined, high-fat diet (CDAHFD) using liquid chromatography/mass spectrometry (LC/MS). An in vitro experiment was performed using HepG2 cells stimulated with recombinant human TNFα or IL1β. The expression of steatosis-, inflammation-, and fibrosis-related genes were analyzed. Plasma samples from NAFLD patients were also analyzed by LC/MS. The CDAHFD-fed db/db mice with hepatic steatosis, inflammation, mild fibrosis, obesity, and hypercholesterolemia displayed significantly higher hepatic and plasma levels of free adrenic acid (p < 0.05). The accumulated adrenic acid in the CDAHFD-fed db/db mice was associated with increased expression of ELOVL2 and 5, and the suppression of the acyl-CoA oxidase 1 gene during peroxisomal β-oxidation. The pretreatment of HepG2 cells with adrenic acid enhanced their cytokine-induced cytokines and chemokines mRNA expression. In NAFLD patients, the group with the highest ALT levels exhibited higher plasma adrenic acid concentrations than the other ALT groups (p-value for trend <0.001). Data obtained demonstrated that adrenic acid accumulation contributes to disease progression in NAFLD. Copyright © 2017 Elsevier Inc. All rights reserved.

From Lysosomal Storage Diseases to NKT Cell Activation and Back

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Cátia S. Pereira

2017-02-01

Full Text Available Lysosomal storage diseases (LSDs are inherited metabolic disorders characterized by the accumulation of different types of substrates in the lysosome. With a multisystemic involvement, LSDs often present a very broad clinical spectrum. In many LSDs, alterations of the immune system were described. Special emphasis was given to Natural Killer T (NKT cells, a population of lipid-specific T cells that is activated by lipid antigens bound to CD1d (cluster of differentiation 1 d molecules at the surface of antigen-presenting cells. These cells have important functions in cancer, infection, and autoimmunity and were altered in a variety of LSDs’ mouse models. In some cases, the observed decrease was attributed to defects in either lipid antigen availability, trafficking, processing, or loading in CD1d. Here, we review the current knowledge about NKT cells in the context of LSDs, including the alterations detected, the proposed mechanisms to explain these defects, and the relevance of these findings for disease pathology. Furthermore, the effect of enzyme replacement therapy on NKT cells is also discussed.

Glycogen storage disease type Ia in canines: a model for human metabolic and genetic liver disease.

Science.gov (United States)

Specht, Andrew; Fiske, Laurie; Erger, Kirsten; Cossette, Travis; Verstegen, John; Campbell-Thompson, Martha; Struck, Maggie B; Lee, Young Mok; Chou, Janice Y; Byrne, Barry J; Correia, Catherine E; Mah, Cathryn S; Weinstein, David A; Conlon, Thomas J

2011-01-01

A canine model of Glycogen storage disease type Ia (GSDIa) is described. Affected dogs are homozygous for a previously described M121I mutation resulting in a deficiency of glucose-6-phosphatase-α. Metabolic, clinicopathologic, pathologic, and clinical manifestations of GSDIa observed in this model are described and compared to those observed in humans. The canine model shows more complete recapitulation of the clinical manifestations seen in humans including "lactic acidosis", larger size, and longer lifespan compared to other animal models. Use of this model in preclinical trials of gene therapy is described and briefly compared to the murine model. Although the canine model offers a number of advantages for evaluating potential therapies for GSDIa, there are also some significant challenges involved in its use. Despite these challenges, the canine model of GSDIa should continue to provide valuable information about the potential for generating curative therapies for GSDIa as well as other genetic hepatic diseases.

Glycogen Storage Disease Type Ia in Canines: A Model for Human Metabolic and Genetic Liver Disease

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Andrew Specht

2011-01-01

Full Text Available A canine model of Glycogen storage disease type Ia (GSDIa is described. Affected dogs are homozygous for a previously described M121I mutation resulting in a deficiency of glucose-6-phosphatase-α. Metabolic, clinicopathologic, pathologic, and clinical manifestations of GSDIa observed in this model are described and compared to those observed in humans. The canine model shows more complete recapitulation of the clinical manifestations seen in humans including “lactic acidosis”, larger size, and longer lifespan compared to other animal models. Use of this model in preclinical trials of gene therapy is described and briefly compared to the murine model. Although the canine model offers a number of advantages for evaluating potential therapies for GSDIa, there are also some significant challenges involved in its use. Despite these challenges, the canine model of GSDIa should continue to provide valuable information about the potential for generating curative therapies for GSDIa as well as other genetic hepatic diseases.

Microbiological and physicochemical profile of traditional Salsiccia toscana during storage

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Giovanna Preziuso

2012-07-01

Full Text Available The aim of this study was to evaluate the evolution during refrigerated storage of the main microbiological and physicochemical parameters of a traditional Italian fresh sausage, salsiccia toscana. The following parameters were analyzed at Days 0, 6 and 9 of storage: total aerobic mesophilic and psychrotrophic counts, Brochothrix thermosphacta, lactic acid bacteria, Pseudomonadaceae, Enterobacteriaceae, Escherichia coli, Micrococcaceae, yeasts and moulds, Salmonella spp., Listeria monocytogenes; pH, Aw, moisture, protein, total lipids, ash, fatty acid composition, Thiobarbituric Acid Reactive Substances, colour measurement. The results revealed a statistically significant increase in the main microbiological parameters between Days 0 and 9 of refrigerated storage, with total bacterial counts exceeding 107 CFU/g after nine days and Brochothrix thermosphacta representing the main spoilage microorganism. Among physicochemical parameters, discolouration was noted during the time of storage, with a significantly paler colour on the surface of sausages with casings after nine days of storage. Considering that the initial bacterial counts were higher than 105 CFU/g for Brochothrix thermosphacta, Pseudomonadaceae, lactic acid bacteria and Enterobacteriaceae, and that the product is traditionally purchased loose without any packaging, it is fundamental to improve the good manufacturing practices, particularly measures to control processing temperatures.

Neural stem cells for disease modeling of Wolman disease and evaluation of therapeutics

OpenAIRE

Aguisanda, Francis; Yeh, Charles D.; Chen, Catherine Z.; Li, Rong; Beers, Jeanette; Zou, Jizhong; Thorne, Natasha; Zheng, Wei

2017-01-01

Background Wolman disease (WD) is a rare lysosomal storage disorder that is caused by mutations in the LIPA gene encoding lysosomal acid lipase (LAL). Deficiency in LAL function causes accumulation of cholesteryl esters and triglycerides in lysosomes. Fatality usually occurs within the first year of life. While an enzyme replacement therapy has recently become available, there is currently no small-molecule drug treatment for WD. Results We have generated induced pluripotent stem cells (iPSCs...

Сhanges in consumer properties of bracken using different storage methods

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I. V. Shalisko

2016-01-01

Full Text Available The article compares the changes in consumer properties of bracken ordinary young shoots at different storage methods. Bracken is a source of vitamins, minerals, it is eaten in some countries -Japan, China, Korea, and also in Russia Shows some advantages in frozen storage. The structural and mechanical changes at themacro and micro levels. Compares elasticity raw samples at different storage methods. Data on the content of amino acids, fatty acids, vitamins, salt, water activity. From the obtained results revealed that the amino acid content in the composition of the frozen bracken decreased significantly as compared to fresh and salt fern (except aspartic acid, isoleucine, and leucine, whose number is close to their number in the salty fern. When storing the fern frozen there is a considerable amount of lipids. Also a significant presence of fat-tion of acids (as % of value, ±10% palmitic (24,86%, hexadecanoate (2,33%, stearic (1,06%, oleic (4,71%, linoleic (26,02%, α-linolenic (11,88%, γ-linolenic (3,13%, dihomo-γ-linolenic (2,28%, arahidonova (0,78%, arachidonic (14,83%, timonova (0,92%, Baganova (0,96%. There is the presence of essential fatty acids. Myristic, berestyeneva, palmitoleate, zonvakantie, andonova acids are present in smaller amount benefits. The results of determination of vitamins in the frozen samples showed that, despite significant losses in storage, they manage to keep the vitamins. It proposed the introduction of the freeze as an effective method of storage bracken. Storage method shoots bracken almost not been studied in frozen form, and it may be that innovation, which will expand the practical use of this type of native raw materials.

The amino acid's backup bone - storage solutions for proteomics facilities.

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Meckel, Hagen; Stephan, Christian; Bunse, Christian; Krafzik, Michael; Reher, Christopher; Kohl, Michael; Meyer, Helmut Erich; Eisenacher, Martin

2014-01-01

Proteomics methods, especially high-throughput mass spectrometry analysis have been continually developed and improved over the years. The analysis of complex biological samples produces large volumes of raw data. Data storage and recovery management pose substantial challenges to biomedical or proteomic facilities regarding backup and archiving concepts as well as hardware requirements. In this article we describe differences between the terms backup and archive with regard to manual and automatic approaches. We also introduce different storage concepts and technologies from transportable media to professional solutions such as redundant array of independent disks (RAID) systems, network attached storages (NAS) and storage area network (SAN). Moreover, we present a software solution, which we developed for the purpose of long-term preservation of large mass spectrometry raw data files on an object storage device (OSD) archiving system. Finally, advantages, disadvantages, and experiences from routine operations of the presented concepts and technologies are evaluated and discussed. This article is part of a Special Issue entitled: Computational Proteomics in the Post-Identification Era. Guest Editors: Martin Eisenacher and Christian Stephan. Copyright © 2013. Published by Elsevier B.V.

Clinical implications of eicosapentaenoic acid/arachidonic acid ratio (EPA/AA) in adult patients with congenital heart disease.

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Kanoh, Miki; Inai, Kei; Shinohara, Tokuko; Tomimatsu, Hirofumi; Nakanishi, Toshio

2017-12-01

Recent studies showed that a low ratio between the levels of eicosapentaenoic acid and those of arachidonic acid (EPA/AA) is associated with higher incidence of coronary artery disease and poor prognosis of heart failure, arrhythmia, and cardiac sudden death. However, the clinical implications of EPA/AA in adult patients with congenital heart disease remain unclear. We aimed to assess the prognostic value of EPA/AA regarding cardiac events in adult patients with congenital heart disease. We measured the serum levels of eicosapentaenoic acid and arachidonic acid in 130 adult patients (median age, 31 years) stratified into two groups according to their EPA/AA (low, ≤0.22; high, >0.22). We prospectively analyzed the association between EPA/AA and incidence of cardiac events during a mean observation period of 15 months, expressed in terms of hazard ratio (HR) with 95% confidence interval (95% CI). In the subgroup of patients with biventricular circulation (2VC) (n = 76), we analyzed the same clinical endpoints. In our study population, EPA/AA was not associated with the incidence of arrhythmic events (HR, 1.52; 95% CI, 0.82-2.85; p = 0.19), but low EPA/AA was a predictor of heart failure hospitalization (HR, 2.83; 95% CI, 1.35-6.30; p AA of ≤0.25 was associated with a significantly higher risk of arrhythmic events (HR, 2.55; 95% CI, 1.11-6.41; p = 0.03) and heart failure hospitalization (HR, 5.20; 95% CI, 1.78-18.1; p AA represents a useful predictor of cardiac events in adult patients with congenital heart disease.

Pompe disease: A case report

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Abdullah Çim

2015-12-01

Full Text Available Pompe disease is inherited in an autosomal recessive manner, and is usually observed in the children of asymptomatic carriers. Pompe disease, known as Glycogen Storage Disorder type II, is caused by pathogenic mutations in the gene encoding lysosomal acid alpha-glucosidase (GAA. There are three types of Pompe disease: classical infantile form, non-classical infantile form and late-onset Pompe disease. Age of onset and severity of the disease determine the type of Pompe disease. We aimed to identify a mutation in GAA gene in parents who were first cousins and their baby girl was passed away due to the Pompe disease. The baby girl had reduced acid alpha-glucosidase activity, but genetic analysis had not been performed. Mutation analysis of parents was performed using high-throughput DNA sequencing method. Heterozygous mutation of c.896 T>C in exon 5 was found in parents, and prenatal diagnosis was performed for their next pregnancy. In conclusion, c.896 T>C substitution in GAA gene may lead to the severe type of Pompe disease. Using a relatively fast and reliable molecular genetic analysis method to confirm the early diagnosis of the Pompe disease is important for the management of the disease.

Omega-3 fatty acids: potential role in the management of early Alzheimer’s disease

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Gregory A Jicha

2010-03-01

Full Text Available Gregory A Jicha, William R MarkesberyUniversity of Kentucky Alzheimer’s Disease Center and the Sanders-Brown Center on Aging University of Kentucky College of Medicine, Lexington, KY, USAbstract: Omega-3 fatty acids are essential for brain growth and development. They play an important role throughout life, as critical modulators of neuronal function and regulation of oxidative stress mechanisms, in brain health and disease. Docosahexanoic acid (DHA, the major omega-3 fatty acid found in neurons, has taken on a central role as a target for therapeutic intervention in Alzheimer’s disease (AD. A plethora of in vitro, animal model, and human data, gathered over the past decade, highlight the important role DHA may play in the development of a variety of neurological and psychiatric disorders, including AD. Cross sectional and prospective cohort data have demonstrated that reduced dietary intake or low brain levels of DHA are associated with accelerated cognitive decline or the development of incipient dementia, including AD. Several clinical trials investigating the effects of omega-3 fatty acid supplementation in AD have been completed and all failed to demonstrate its efficacy in the treatment of AD. However, these trials produced intriguing data suggesting that the beneficial effects of omega-3 fatty acid supplementation may depend on the stage of disease, other dietary mediators, and apolipoprotein E status.Keywords: Alzheimer’s disease, omega-3 fatty acids, oxidative stress, clinical studies, treatment

Use of modified cornstarch therapy to extend fasting in glycogen storage disease types Ia and Ib

NARCIS (Netherlands)

Correia, Catherine E.; Bhattacharya, Kaustuv; Lee, Philip J.; Shuster, Jonathan J.; Theriaque, Douglas W.; Shankar, Meena N.; Smit, G. Peter A.; Weinstein, David A.

2008-01-01

Background: Type I glycogen storage disease (GSD) is caused by a deficiency of glucose-6-phosphatase resulting in severe fasting hypoglycemia. Objective: We compared the efficacy of a new modified starch with the currently used cornstarch therapy in patients with type Ia and Ib GSD. Design: This was

Comparative proteomic and metabolomic profiling of citrus fruit with enhancement of disease resistance by postharvest heat treatment.

Science.gov (United States)

Yun, Ze; Gao, Huijun; Liu, Ping; Liu, Shuzhen; Luo, Tao; Jin, Shuai; Xu, Qiang; Xu, Juan; Cheng, Yunjiang; Deng, Xiuxin

2013-03-16

From field harvest to the consumer's table, fresh citrus fruit spends a considerable amount of time in shipment and storage. During these processes, physiological disorders and pathological diseases are the main causes of fruit loss. Heat treatment (HT) has been widely used to maintain fruit quality during postharvest storage; however, limited molecular information related to this treatment is currently available at a systemic biological level. Mature 'Kamei' Satsuma mandarin (Citrus unshiu Marc.) fruits were selected for exploring the disease resistance mechanisms induced by HT during postharvest storage. Proteomic analyses based on two-dimensional gel electrophoresis (2-DE), and metabolomic research based on gas chromatography coupled to mass spectrometry (GC-MS), and liquid chromatography quadrupole time-of-flight mass spectrometry (LC-QToF-MS) were conducted. The results show resistance associated proteins were up-regulated in heat treated pericarp, such as beta-1, 3-glucanase, Class III chitinase, 17.7 kDa heat shock protein and low molecular weight heat-shock protein. Also, redox metabolism enzymes were down-regulated in heat treated pericarp, including isoflavone reductase, oxidoreductase and superoxide dismutase. Primary metabolic profiling revealed organic acids and amino acids were down-regulated in heat treated pericarp; but significant accumulation of metabolites, including tetradecanoic acid, oleic acid, ornithine, 2-keto-d-gluconic acid, succinic acid, turanose, sucrose, galactose, myo-inositol, glucose and fructose were detected. Noticeably, H2O2 content decreased, while, lignin content increased in heat treated pericarp compared to the control, which might increase fruit resistibility in response to external stress. Also, flavonoids, substances which are well-known to be effective in reducing external stress, were up-regulated in heat treated pericarp. This study provides a broad picture of differential accumulation of proteins and metabolites in

Investigation and management of the hepatic glycogen storage diseases.

Science.gov (United States)

Bhattacharya, Kaustuv

2015-07-01

The glycogen storage diseases (GSD) comprise a group of disorders that involve the disruption of metabolism of glycogen. Glycogen is stored in various organs including skeletal muscle, the kidneys and liver. The liver stores glycogen to supply the rest of the body with glucose when required. Therefore, disruption of this process can lead to hypoglycaemia. If glycogen is not broken down effectively, this can lead to hepatomegaly. Glycogen synthase deficiency leads to impaired glycogen synthesis and consequently the liver is small. Glycogen brancher deficiency can lead to abnormal glycogen being stored in the liver leading to a quite different disorder of progressive liver dysfunction. Understanding the physiology of GSD I, III, VI and IX guides dietary treatments and the provision of appropriate amounts and types of carbohydrates. There has been recent re-emergence in the literature of the use of ketones in therapy, either in the form of the salt D,L-3-hydroxybutyrate or medium chain triglyceride (MCT). High protein diets have also been advocated. Alternative waxy maize based starches seem to show promising early data of efficacy. There are many complications of each of these disorders and they need to be prospectively surveyed and managed. Liver and kidney transplantation is still indicated in severe refractory disease.

Multiple-unit tablet of probiotic bacteria for improved storage stability, acid tolerability, and in vivo intestinal protective effect

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Park HJ

2016-04-01

Full Text Available Hee Jun Park,1 Ga Hyeon Lee,1 Joonho Jun,1 Miwon Son,1 Myung Joo Kang2 1Dong-A Pharmaceutical Co. Ltd., Yongin, Gyeonggi, 2College of Pharmacy, Dankook University, Cheonan, Chungnam, Korea Abstract: The aim of this study was to formulate probiotics-loaded pellets in a tablet form to improve storage stability, acid tolerability, and in vivo intestinal protective effect. Bacteria-loaded pellets primarily prepared with hydroxypropyl methylcellulose acetate succinate were compressed into tablets with highly compressible excipients and optimized for flow properties, hardness, and disintegration time. The optimized probiotic tablet consisted of enteric-coated pellets (335 mg, microcrystalline cellulose (Avicel PH102, 37.5 mg, and porous calcium silicate (25 mg and allowed whole survival of living bacteria during the compaction process with sufficient tablet hardness (13 kp and disintegration time (14 minutes. The multiple-unit tablet showed remarkably higher storage stability under ambient conditions (25°C/60% relative humidity over 6 months and resistance to acidic medium compared to uncoated strains or pellets. Repeated intake of this multiple-unit tablet significantly lowered plasma level of endotoxin, a pathogenic material, compared to repeated intake of bare probiotics or marketed products in rats. These results, therefore, suggest that the multiple-unit tablet is advantageous to better bacterial viability and gain the beneficial effects on the gut flora, including the improvement of intestinal barrier function. Keywords: probiotics, multiple-unit tablet, bacterial viability, acid resistance, intestinal barrier function

Glycogen storage disease type I: clinical and laboratory profile

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Berenice L. Santos

2014-11-01

Full Text Available Objectives: To characterize the clinical, laboratory, and anthropometric profile of a sample of Brazilian patients with glycogen storage disease type I managed at an outpatient referral clinic for inborn errors of metabolism. Methods: This was a cross-sectional outpatient study based on a convenience sampling strategy. Data on diagnosis, management, anthropometric parameters, and follow-up were assessed. Results: Twenty-one patients were included (median age 10 years, range 1–25 years, all using uncooked cornstarch therapy. Median age at diagnosis was 7 months (range, 1–132 months, and 19 patients underwent liver biopsy for diagnostic confirmation. Overweight, short stature, hepatomegaly, and liver nodules were present in 16 of 21, four of 21, nine of 14, and three of 14 patients, respectively. A correlation was found between height-for-age and BMI-for-age Z-scores (r = 0.561; p = 0.008. Conclusions: Diagnosis of glycogen storage disease type I is delayed in Brazil. Most patients undergo liver biopsy for diagnostic confirmation, even though the combination of a characteristic clinical presentation and molecular methods can provide a definitive diagnosis in a less invasive manner. Obesity is a side effect of cornstarch therapy, and appears to be associated with growth in these patients. Resumo: Objetivos: Caracterizar o perfil clínico, laboratorial e antropométrico de uma amostra de pacientes brasileiros com doença de depósito de glicogênio tipo I tratados em um ambulatório de referência para erros inatos do metabolismo. Métodos: Este foi um estudo ambulatorial transversal com base em uma estratégia de amostragem de conveniência. Foram avaliados os dados com relação ao diagnóstico, tratamento, parâmetros antropométricos e acompanhamento. Resultados: Foram incluídos 21 pacientes (idade média de 10 anos, faixa 1-25 anos de idade, e todos se encontravam em terapia de amido de milho cru. A idade média na época do diagn

Effect of ursodeoxycholic acid on the kinetics of the major hydrophobic bile acids in health and in chronic cholestatic liver disease

NARCIS (Netherlands)

Beuers, U.; Spengler, U.; Zwiebel, F. M.; PAULETZKI, J.; Fischer, S.; Paumgartner, G.

1992-01-01

Beneficial effects of ursodeoxycholic acid in chronic cholestatic liver diseases have been attributed to displacement of hydrophobic bile acids from the endogenous bile acid pool. To test this hypothesis, we determined pool sizes, fractional turnover rates, synthesis/input rates and serum levels of

Temperature distributions in trapezoidal built in storage solar water heaters with/without phase change materials

International Nuclear Information System (INIS)

Tarhan, Sefa; Sari, Ahmet; Yardim, M. Hakan

2006-01-01

Built in storage solar water heaters (BSSWHs) have been recognized for their more compact constructions and faster solar gain than conventional solar water heaters, however, their water temperatures quickly go down during the cooling period. A trapezoidal BSSWH without PCM storage unit was used as the control heater (reference) to investigate the effect of two differently configured PCM storage units on the temperature distributions in water tanks. In the first design, myristic acid was filled into the PCM storage tank, which also served as an absorbing plate. In the second design, lauric acid was filled into the PCM storage tank, which also served as a baffle plate. The water temperature changes were followed by five thermocouples placed evenly and longitudinally into each of the three BSSWHs. The effects of the PCMs on the water temperature distributions depended on the configuration of the PCM storage unit and the longitudinal position in the water tanks. The use of lauric acid lowered the values of the peak temperatures by 15% compared to the control heater at the upper portion of the water tanks because of the low melting temperature of lauric acid, but it did not have any consistent effect on the retention of the water temperatures during the cooling period. The ability of the myristic acid storage unit to retain the water temperatures got more remarkable, especially at the middle portion of the water tank. The myristic acid storage increased the dip temperatures by approximately 8.8% compared to the control heater. In conclusion, lauric acid storage can be used to stabilize the water temperature during the day time, while the myristic acid storage unit can be used as a thermal barrier against heat loss during the night time because of its relatively high melting temperature and low heat conduction coefficient in its solid phase. The experimental results have also indicated that the thermal characteristics of the PCM and the configuration of the PCM storage

Acid and non-acid reflux patterns in patients with erosive esophagitis and non-erosive reflux disease (NERD): a study using intraluminal impedance monitoring

NARCIS (Netherlands)

Conchillo, José M.; Schwartz, Matthijs P.; Selimah, Mohamed; Samsom, Melvin; Sifrim, Daniel; Smout, André J.

2008-01-01

BACKGROUND: Non-erosive reflux disease (NERD) and erosive esophagitis (EE) are the most common phenotypic presentations of gastroesophageal reflux disease (GERD). AIM: To assess acid and non-acid reflux patterns in patients with EE and NERD using combined esophageal pH-impedance monitoring. METHODS:

Acid and non-acid reflux patterns in patients with erosive esophagitis and non-erosive reflux disease (NERD) : A study using intraluminal impedance monitoring

NARCIS (Netherlands)

Conchillo, Jose M.; Schwartz, Matthijs P.; Selimah, Mohamed; Samsom, Melvin; Sifrim, Daniel; Smout, Andre J.

Background Non-erosive reflux disease (NERD) and erosive esophagitis (EE) are the most common phenotypic presentations of gastroesophageal reflux disease (GERD). Aim To assess acid and non-acid reflux patterns in patients with EE and NERD using combined esophageal pH-impedance monitoring. Methods A

Effect of holding period prior to storage on the chemical attributes of Starking Delicious apples during refrigerated storage

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Aynur Batkan

2012-06-01

Full Text Available In this research, the effects of three different holding periods (6, 12 and 24 hours prior to storage on the quality attributes of Starking Delicious apples were investigated during storage of 8 months at 0.5 ± 1.0 ºC. Changes in weight loss, flesh firmness, pH values, soluble dry matter amount, titratable acidity values, ascorbic acid contents, and total and reducing sugar content were determined. According to the results, the holding period showed statistically significant changes in the quality attributes of the apples (p < 0.05.

Long-term storage and safe retrieval of human papillomavirus DNA using FTA elute cards.

Science.gov (United States)

Barth, Heidi; Morel, Adrien; Mougin, Christiane; Averous, Gerlinde; Legrain, Michèle; Fender, Muriel; Risch, Simone; Fafi-Kremer, Samira; Velten, Michel; Oudet, Pierre; Baldauf, Jean-Jacques; Stoll-Keller, Françoise

2016-03-01

Biobanking or collection and storage of specimens for future research purposes have become an essential tool in many fields of biomedical research and aims to provide a better understanding of disease mechanisms as well as the identification of disease-specific biomarkers that can navigate in complex diseases. In this study, we assessed the use of Flinders Technology Associates (FTA) cards as a long-term storage device for cervical specimens with suspected human papillomavirus (HPV) infections. HPV detection and genotyping results in liquid-based transport media were compared to HPV results from FTA cards. The overall agreement for the presence of any HPV infection between liquid-based medium and FTA cards stored for 1 year at ambient temperature was 100%. Reproducibility analysis of HPV detection and genotyping from FTA cards demonstrated that FTA cards are a reliable medium to store and preserve viral nucleic acids. Biobanking of cervical cells on FTA cards may provide a key resource for epidemiological and retrospective HPV studies. Copyright © 2015 Elsevier B.V. All rights reserved.

Synthesis and characterization of microencapsulated myristic acid–palmitic acid eutectic mixture as phase change material for thermal energy storage

International Nuclear Information System (INIS)

Alva, Guruprasad; Huang, Xiang; Liu, Lingkun; Fang, Guiyin

2017-01-01

Highlights: •Myristic acid–palmitic acid eutectic was microencapsulated with silica shell. •Structure, morphology of microencapsulated phase change material were investigated. •Thermal capacity, stability of microencapsulated phase change material were analyzed. •Silica shell improved thermal stability of microencapsulated phase change material. -- Abstract: In this work microencapsulation of myristic acid–palmitic acid (MA–PA) eutectic mixture with silica shell using sol−gel method has been attempted. The core phase change material (PCM) for thermal energy storage was myristic acid−palmitic acid eutectic mixture and the shell material to prevent the PCM core from leakage was silica prepared from methyl triethoxysilane (MTES). Thermal properties of the microcapsules were measured by differential scanning calorimeter (DSC). The morphology and particle size of the microcapsules were examined by scanning electronic microscope (SEM). Fourier transformation infrared spectrophotometer (FT–IR) and X–ray diffractometer (XRD) were used to investigate the chemical structure and crystalloid phase of the microcapsules respectively. The DSC results indicated that microencapsulated phase change material (MPCM) melts at 46.08 °C with a latent heat of 169.69 kJ kg −1 and solidifies at 44.35 °C with a latent heat of 159.59 kJ kg −1 . The thermal stability of the microcapsules was analyzed by a thermogravimeter (TGA). The results indicated that the MPCM has good thermal stability and is suitable for thermal energy storage application.

Oleanolic Acid and Its Derivatives: Biological Activities and Therapeutic Potential in Chronic Diseases

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Taiwo Betty Ayeleso

2017-11-01

Full Text Available The increasing demand for natural products as an alternative therapy for chronic diseases has encouraged research into the pharmacological importance of bioactive compounds from plants. Recently, there has been a surge of interest in the therapeutic potential of oleanolic acid (OA in the prevention and management of chronic diseases. Oleanolic acid is a pentacyclic triterpenoid widely found in plants, including fruits and vegetables with different techniques and chromatography platforms being employed in its extraction and isolation. Several studies have demonstrated the potential therapeutic effects of OA on different diseases and their symptoms. Furthermore, oleanolic acid also serves as a framework for the development of novel semi-synthetic triterpenoids that could prove vital in finding therapeutic modalities for various ailments. There are recent advances in the design and synthesis of chemical derivatives of OA to enhance its solubility, bioavailability and potency. Some of these derivatives have also been therapeutic candidates in a number of clinical trials. This review consolidates and expands on recent reports on the biological effects of oleanolic acid from different plant sources and its synthetic derivatives as well as their mechanisms of action in in vitro and in vivo study models. This review suggests that oleanolic acid and its derivatives are important candidates in the search for alternative therapy in the treatment and management of chronic diseases.

A novel PNPLA2 mutation causes neutral lipid storage disease with myopathy (NLSDM) presenting muscular dystrophic features with lipid storage and rimmed vacuoles.

Science.gov (United States)

Chen, J; Hong, D; Wang, Z; Yuan, Y

2010-01-01

Neutral lipid storage disease with myopathy (NLSDM) is a type of lipid storage myopathy arising due to a mutation in the PNPLA2 gene encoding an adipose triglyceride lipase responsible for the degradation of intracellular triglycerides. Herein, we report the cases of two siblings manifesting slowly progressive proximal and distal limb weakness in adulthood. One of the patients had dilated cardiomyopathy, hearing loss and short stature. Muscle specimens of the 2 patients revealed muscular dystrophic features with massive lipid droplets and numerous rimmed vacuoles in the fibers. A novel homozygous mutation IVS2+1G > A in the PNPLA2 gene was identified in the 2 cases, but not in the healthy familial individuals. The presence of massive lipid droplets with muscular dystrophic changes and rimmed vacuoles in muscle fibers might be one of the characteristic pathological changes of NLSDM.

Fluorogenic Substrates for Visualizing Acidic Organelle Enzyme Activities.

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Fiona Karen Harlan

Full Text Available Lysosomes are acidic cytoplasmic organelles that are present in all nucleated mammalian cells and are involved in a variety of cellular processes including repair of the plasma membrane, defense against pathogens, cholesterol homeostasis, bone remodeling, metabolism, apoptosis and cell signaling. Defects in lysosomal enzyme activity have been associated with a variety of neurological diseases including Parkinson's Disease, Lysosomal Storage Diseases, Alzheimer's disease and Huntington's disease. Fluorogenic lysosomal staining probes were synthesized for labeling lysosomes and other acidic organelles in a live-cell format and were shown to be capable of monitoring lysosomal metabolic activity. The new targeted substrates were prepared from fluorescent dyes having a low pKa value for optimum fluorescence at the lower physiological pH found in lysosomes. They were modified to contain targeting groups to direct their accumulation in lysosomes as well as enzyme-cleavable functions for monitoring specific enzyme activities using a live-cell staining format. Application to the staining of cells derived from blood and skin samples of patients with Metachromatic Leukodystrophy, Krabbe and Gaucher Diseases as well as healthy human fibroblast and leukocyte control cells exhibited localization to the lysosome when compared with known lysosomal stain LysoTracker® Red DND-99 as well as with anti-LAMP1 Antibody staining. When cell metabolism was inhibited with chloroquine, staining with an esterase substrate was reduced, demonstrating that the substrates can be used to measure cell metabolism. When applied to diseased cells, the intensity of staining was reflective of lysosomal enzyme levels found in diseased cells. Substrates specific to the enzyme deficiencies in Gaucher or Krabbe disease patient cell lines exhibited reduced staining compared to that in non-diseased cells. The new lysosome-targeted fluorogenic substrates should be useful for research

Impaired autophagy in the lipid-storage disorder Niemann-Pick type C1 disease.

Science.gov (United States)

Sarkar, Sovan; Carroll, Bernadette; Buganim, Yosef; Maetzel, Dorothea; Ng, Alex H M; Cassady, John P; Cohen, Malkiel A; Chakraborty, Souvik; Wang, Haoyi; Spooner, Eric; Ploegh, Hidde; Gsponer, Joerg; Korolchuk, Viktor I; Jaenisch, Rudolf

2013-12-12

Autophagy dysfunction has been implicated in misfolded protein accumulation and cellular toxicity in several diseases. Whether alterations in autophagy also contribute to the pathology of lipid-storage disorders is not clear. Here, we show defective autophagy in Niemann-Pick type C1 (NPC1) disease associated with cholesterol accumulation, where the maturation of autophagosomes is impaired because of defective amphisome formation caused by failure in SNARE machinery, whereas the lysosomal proteolytic function remains unaffected. Expression of functional NPC1 protein rescues this defect. Inhibition of autophagy also causes cholesterol accumulation. Compromised autophagy was seen in disease-affected organs of Npc1 mutant mice. Of potential therapeutic relevance is that HP-β-cyclodextrin, which is used for cholesterol-depletion treatment, impedes autophagy, whereas stimulating autophagy restores its function independent of amphisome formation. Our data suggest that a low dose of HP-β-cyclodextrin that does not perturb autophagy, coupled with an autophagy inducer, may provide a rational treatment strategy for NPC1 disease. Copyright © 2013 The Authors. Published by Elsevier Inc. All rights reserved.

The acid pocket: a target for treatment in reflux disease?

Science.gov (United States)

Kahrilas, Peter J; McColl, Kenneth; Fox, Mark; O'Rourke, Lisa; Sifrim, Daniel; Smout, Andre J P M; Boeckxstaens, Guy

2013-07-01

The nadir esophageal pH of reflux observed during pH monitoring in the postprandial period is often more acidic than the concomitant intragastric pH. This paradox prompted the discovery of the "acid pocket", an area of unbuffered gastric acid that accumulates in the proximal stomach after meals and serves as the reservoir for acid reflux in healthy individuals and gastroesophageal reflux disease (GERD) patients. However, there are differentiating features between these populations in the size and position of the acid pocket, with GERD patients predisposed to upward migration of the proximal margin onto the esophageal mucosa, particularly when supine. This upward migration of acid, sometimes referred to as an "acid film", likely contributes to mucosal pathology in the region of the squamocolumnar junction. Furthermore, movement of the acid pocket itself to a supradiaphragmatic location with hiatus hernia increases the propensity for acid reflux by all conventional mechanisms. Consequently, the acid pocket is an attractive target for GERD therapy. It may be targeted in a global way with proton pump inhibitors that attenuate acid pocket development, or with alginate/antacid combinations that colocalize with the acid pocket and displace it distally, thereby demonstrating the potential for selective targeting of the acid pocket in GERD.

[Fatty acids in mature breast milk from low socioeconomic levels of Venezuelan women: influence of temperature and time of storage].

Science.gov (United States)

Bosch, Virgilio; Golfetto, Iván; Alonso, Hilda; Laurentin, Zuly; Materan, Mercedes; García, Ninoska

2009-03-01

Fatty acids in mature breast milk from low socioeconomic levels of Venezuelan women: influence of temperature and time of storage. Breast milk is the main food in infants from birth until six months old. It is important to know if precarious life conditions could limit some nutrients in mother's milk. The objective of this study is to evaluate the total fat and essential long chain fatty acids in mature breast milk from low socioeconomic levels in Venezuelan women. The values of total fat (3.56 +/- 1.18 g/%) are similar that reported in the literature, however the sume of LC-PUFA n-3 was 0.3 +/- 0.04% which is related whith low n-3 fatty acid maternal diet.The sume LC-PUFA n-3 contained in this study is below most of the reviewed publications. The average amount of 22:6 n-3 in breast milk offered to newborn one month old (750 ml/day) is below estimated requirements (70 mg/day). The majority of these samples provide to the infants, the amount of DHA estimated as convenient to sustain normal growth. Also it was explored how the time (8h to 24 h) and temperatura (+4 degrees C, +15 degrees C, and +25 degrees C) can affect its composition. This data will permit to select the best condiitions of sampling and storage of mother's milk in future investigations in different regions of Venezuela. Most of the breast milk fatty acids tolerate some hours at room temperature (25 degrees C) but essential long chain fatty acids are very vulnerable. We propose that, in consequence, that samples should be transported in sterile conditions in dry ice to the laboratory in a few hours and should be kept at -70 degrees C until their analysis.

Liver fibrosis in type I Gaucher disease: magnetic resonance imaging, transient elastography and parameters of iron storage.

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Anneloes E Bohte

Full Text Available Long term liver-related complications of type-1 Gaucher disease (GD, a lysosomal storage disorder, include fibrosis and an increased incidence of hepatocellular carcinoma. Splenectomy has been implicated as a risk factor for the development of liver pathology in GD. High ferritin concentrations are a feature of GD and iron storage in Gaucher cells has been described, but iron storage in the liver in relation to liver fibrosis has not been studied. Alternatively, iron storage in GD may be the result of iron supplementation therapy or regular blood transfusions in patients with severe cytopenia. In this pilot study, comprising 14 type-1 GD patients (7 splenectomized, 7 non-splenectomized and 7 healthy controls, we demonstrate that liver stiffness values, measured by Transient Elastography and MR-Elastography, are significantly higher in splenectomized GD patients when compared with non-splenectomized GD patients (p = 0.03 and p = 0.01, respectively. Liver iron concentration was elevated (>60±30 µmol/g in 4 GD patients of whom 3 were splenectomized. No relationship was found between liver stiffness and liver iron concentration. HFE gene mutations were more frequent in splenectomized (6/7 than in non-splenectomized (2/7 participants (p = 0.10. Liver disease appeared more advanced in splenectomized than in non-splenectomized patients. We hypothesize a relationship with excessive hepatic iron accumulation in splenectomized patients. We recommend that all splenectomized patients, especially those with evidence of substantial liver fibrosis undergo regular screening for HCC, according to current guidelines.

Okadaic acid

DEFF Research Database (Denmark)

Danielsen, E Michael; Hansen, Gert H; Severinsen, Mai C K

2014-01-01

are the hallmark of phospholipidosis, a pathological condition characterized by lysosomal phospholipid accumulation. Phospholipidosis is observed in acquired lysosomal storage diseases and is induced by a large number of cationic amphiphilic drugs. Unlike the latter, however, OA does not act by accumulating...... in acidic organelles, implying a different toxic mechanism of action. We propose that rapid induction of LBs, an indicator of phospholipidosis, should be included in the future toxicity profile of OA....... hyper protein phosphorylation, but no detectable loss of cell polarity or cytoskeletal integrity of the enterocytes. Using a fluorescent membrane marker, FM dye, endocytosis from the brush border was affected by the toxin. Although constitutive uptake into subapical terminal web-localized early...

Uric acid stones increase the risk of chronic kidney disease.

Science.gov (United States)

Li, Ching-Chia; Chien, Tsu-Ming; Wu, Wen-Jeng; Huang, Chun-Nung; Chou, Yii-Her

2018-02-28

The aim of this study was to compare the clinical characteristics of uric acid stones and their potential risk for chronic kidney disease (CKD). A total of 401 patients (196 with uric acid stone and 205 without) were enrolled from our database of patients with urolithiasis. We analyzed the clinical demographic features, stone location, urine chemistries, and renal function. There was a significant difference (p uric acid group. Patients with uric acid stones had much lower pH of urine (p uric acid level (p = 0.002). Notably, those with uric acid stones had worse eGFR than those with non-uric acid stones. Multivariate analysis confirmed that age over 60 years (ORs = 9.19; 95% CI 3.5-24.3), female sex (ORs = 4.01; 95% CI 1.8-9.0), hyperuricemia (ORs = 8.47; 95% CI 1.6-43.5), and uric acid stone (OR = 2.86; 95% CI 1.2-6.7) were the independent predictors of poor prognoses in CKD. Therefore, an association exists between uric acid stones and higher prevalence of CKD. Patients with uric acid stones may need close monitoring of renal function during follow-up.

Nucleic Acid-Based Therapy Approaches for Huntington's Disease

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Tatyana Vagner

2012-01-01

Full Text Available Huntington's disease (HD is caused by a dominant mutation that results in an unstable expansion of a CAG repeat in the huntingtin gene leading to a toxic gain of function in huntingtin protein which causes massive neurodegeneration mainly in the striatum and clinical symptoms associated with the disease. Since the mutation has multiple effects in the cell and the precise mechanism of the disease remains to be elucidated, gene therapy approaches have been developed that intervene in different aspects of the condition. These approaches include increasing expression of growth factors, decreasing levels of mutant huntingtin, and restoring cell metabolism and transcriptional balance. The aim of this paper is to outline the nucleic acid-based therapeutic strategies that have been tested to date.

Light exposure during storage preserving soluble sugar and l-ascorbic acid content of minimally processed romaine lettuce (Lactuca sativa L.var. longifolia).

Science.gov (United States)

Zhan, Lijuan; Hu, Jinqiang; Ai, Zhilu; Pang, Lingyun; Li, Yu; Zhu, Meiyun

2013-01-01

Minimally processed romaine lettuce (MPRL) leaves were stored in light condition (2500lux) or darkness at 4°C for 7d. Light exposure significantly delayed the degradation of chlorophyll and decrease of glucose, reducing sugar, and sucrose content, and thus preserved more total soluble solid (TSS) content at the end of storage in comparison with darkness. While, it did not influenced starch content that progressively decreased over time. The l-ascorbic acid (AA) accumulated in light-stored leaves, but deteriorated in dark-stored leaves during storage. The dehydroascorbic acid (DHA) increased in all leaves stored in both light and dark condition, of which light condition resulted in less DHA than darkness. In addition, the fresh weight loss and dry matter significantly increased and these increases were accelerated by light exposure. Conclusively, light exposure in applied intensity effectively alleviated MPRL quality deterioration by delaying the decreases of pigments, soluble sugar, TSS content and accumulating AA. Copyright © 2012 Elsevier Ltd. All rights reserved.

Sensory Quality of Standard and Light Mayonnaise during Storage

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Romana Karas

2002-01-01

Full Text Available The effects of ingredients on the quality and stability of two kinds of mayonnaise after it has been stored in a refrigerator (t = 5–8 °C and at room temperature (t = 20–25 °C were examined in this research. Fresh mayonnaise samples were examined by sensory analysis (analytical descriptive test and physico-chemical analyses (pH, acid and peroxide values first after being stored for one month and then after two months. The effects of individual factors were examined and the results were statistically valued. The results show that fresh standard mayonnaise containing 75 % of oil as opposed to low-fat mayonnaise (light mayonnaise containing 49 % of oil gained higher grades for most sensory attributes. Storage temperature, storage time and type of mayonnaise influence significantly (P≤0.001 the stability, homogeneity, mouth feeling, acid odour and flavour, rancidity, pH, acid and peroxide values. At any point of storage and under both temperature regimes, the samples of light mayonnaise had lower pH value and higher acid value in comparison with standard mayonnaise. Regardless of storage conditions irrespective of the recipe for their preparation, pH value was declining, whereas acid and peroxide values increased in all types of mayonnaise. Some meaningful statistically significant correlations (r > 0.70 exist between sensory and physico-chemical parametres of mayonnaise.

Electric energy storage - Overview of technologies

International Nuclear Information System (INIS)

Boye, Henri

2013-01-01

Energy storage is a challenging and costly process, as electricity can only be stored by conversion into other forms of energy (e.g. potential, thermal, chemical or magnetic energy). The grids must be precisely balanced in real time and it must be made sure that the cost of electricity is the lowest possible. Storage of electricity has many advantages, in centralized mass storages used for the management of the transmission network, or in decentralized storages of smaller dimensions. This article presents an overview of the storage technologies: mechanical storage in hydroelectric and pumped storage power stations, compressed air energy storage (CAES), flywheels accumulating kinetic energy, electrochemical batteries with various technologies, traditional lead acid batteries, lithium ion, sodium sulfur (NaS) and others, including vehicle to grid, sensible heat thermal storage, superconducting magnetic energy storage (SMES), super-capacitors, conversion into hydrogen... The different technologies are compared in terms of cost and level of maturity. The development of intermittent renewable energies will result in a growing need for mechanisms to regulate energy flow and innovative energy storage solutions seem well positioned to develop. (author)

THE IMPACT OF ENVIRONMENTALLY FRIENDLY POSTHARVEST TREATMENTS ON THE ANTIOXIDANT ACTIVITY OF STRAWBERRY FRUITS DURING STORAGE

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Ivna Štolfa

2014-12-01

Full Text Available Proper postharvest storage is an effective way to maintain the quality and nutritional values of fruits. The aim of this study was to determine how environmentally friendly postharvest treatments with salicylic acid solution, colloidal silver solution and ozone, affect the antioxidant activity of strawberry fruits (Fragaria x ananassa Duch. cv. Albion during 7 days of storage at 4°C. The content of ascorbic acid, total phenols and antioxidant activity of strawberry fruits were determined spec-trophotometrically. After 7 days of storage in strawberry fruits treated with all three treatments separately, the contents of ascorbic acid were higher than in the control fruits, supporting the usefulness of these treatments for preserving fruit quality and nutritional value during storage. The treatment with salicylic acid solution showed the most beneficial effect during storage causing a significant increase in the content of ascorbic acid, phenols and antioxidant activity at the end of the storage period.

Omega-3 fatty acids' supplementation in Alzheimer's disease: A systematic review.

Science.gov (United States)

Canhada, Scheine; Castro, Kamila; Perry, Ingrid Schweigert; Luft, Vivian Cristine

2017-05-03

Alzheimer's disease (AD) is a neurodegeneration disorder characterized by progressive impairments of memory, language, reasoning, and other cognitive functions. Evidence suggests that omega-3 fatty acids may act as a possible protection factor in AD. To evaluate the results available in the literature involving omega-3 fatty acids supplementation and its effect on cognitive function in AD patients. A systematic review of MEDLINE (from PubMed), Excerpta Medica Database, and Cochrane Library databases was conducted according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Inclusion criteria consisted in original intervention studies, controlled by placebo, that assessed the impact of supplementation or dietary intake of omega-3 fatty acids on cognitive function, in humans with AD, without limitation for prime date of publication. Initial search resulted in 361 articles. Seven studies fully met the inclusion criteria. Most studies did not find statistically significant results for the omega-3 fatty acids supplementation compared to placebo, and those who show some benefit do it only in a few cognitive assessment scales. However, the effects of omega-3 fatty acids appear to be most effectively demonstrated in patients with very mild AD. The effects of omega-3 fatty acids supplementation in mild AD corroborate epidemiological observational studies showing that omega-3 fatty acids may be beneficial in disease onset, when there is slight impairment of brain function. Although some studies have shown changes in scales of cognitive function in more severe cases, they are not enough to support omega-3 fatty acids supplementation in the treatment of AD.

Divergent clinical outcomes of alpha-glucosidase enzyme replacement therapy in two siblings with infantile-onset Pompe disease treated in the symptomatic or pre-symptomatic state

OpenAIRE

Matsuoka, Takashi; Miwa, Yoshiyuki; Tajika, Makiko; Sawada, Madoka; Fujimaki, Koichiro; Soga, Takashi; Tomita, Hideshi; Uemura, Shigeru; Nishino, Ichizo; Fukuda, Tokiko; Sugie, Hideo; Kosuga, Motomichi; Okuyama, Torayuki; Umeda, Yoh

2016-01-01

Pompe disease is an autosomal recessive, lysosomal glycogen storage disease caused by acid ?-glucosidase deficiency. Infantile-onset Pompe disease (IOPD) is the most severe form and is characterized by cardiomyopathy, respiratory distress, hepatomegaly, and skeletal muscle weakness. Untreated, IOPD generally results in death within the first year of life. Enzyme replacement therapy (ERT) with recombinant human acid alpha glucosidase (rhGAA) has been shown to markedly improve the life expectan...

The Characteristics of Postprandial Proximal Gastric Acid Pocket in Gastroesophageal Reflux Disease

Science.gov (United States)

Wu, Jing; Liu, Dong; Feng, Cheng; Luo, Yumei; Nian, Yuanyuan; Wang, Xueqin; Zhang, Jun

2018-01-01

Background Postprandial proximal gastric acid pocket (PPGAP) plays important roles in gastroesophageal reflux disease (GERD). In this study, we analyzed the characteristics of PPGAP in GERD. Material/Methods There were 17 normal participants and 20 GERD patients who completed a gastroesophageal reflux disease questionnaire (GerdQ) and underwent a gastroscopy, a high-resolution manometry, an esophageal 24-hour pH monitoring, and a station pull-through pH monitoring to assess their symptomatic degree, endoscopic change, acid exposure, and PPGAP. Results PPGAP was present in all participants. Compared with normal participants, the PPGAP in GERD patients was significantly different, thus the disappearing time was significantly later (pGERD patients was positively correlated with GerdQ score (pGERD patients was positively correlated with the DeMeester score (pGERD patients was negatively correlated with the DeMeester score (pGERD patients had characteristics of long time period, long length, and high acidity. Its length was positively correlated with subjective symptomatic degree. Its period, length, and acidity were positively correlated with the objective acid exposure. PPGAP seems to be the originator of acid reflux events and plays important roles in GERD. PMID:29309401

Human acid β-glucosidase: isolation and amino acid sequence of a peptide containing the catalytic site

International Nuclear Information System (INIS)

Dinur, T.; Osiecki, K.M.; Legler, G.; Gatt, S.; Desnick, R.J.; Grabowski, G.A.

1986-01-01

Human acid β-glucosidase (D-glucosyl-N-acylsphingosine glucohydrolase, EC 3.2.1.45) cleaves the glucosidic bonds of glucosylceramide and synthetic β-glucosides. The deficient activity of this hydrolase is the enzymatic defect in the subtypes and variants of Gaucher disease, the most prevalent lysosomal storage disease. To isolate and characterize the catalytic site of the normal enzyme, brominated 3 H-labeled conduritol B epoxide ( 3 H-Br-CBE), which inhibits the enzyme by binding covalently to this site, was used as an affinity label. Under optimal conditions 1 mol of 3 H-Br-CBE bound to 1 mol of pure enzyme protein, indicating the presence of a single catalytic site per enzyme subunit. After V 8 protease digestion of the 3 H-Br-CBE-labeled homogeneous enzyme, three radiolabeled peptides, designated peptide A, B, or C, were resolved by reverse-phase HPLC. The partial amino acid sequence (37 residues) of peptide A (M/sub r/, 5000) was determined. The sequence of this peptide, which contained the catalytic site, had exact homology to the sequence near the carboxyl terminus of the protein, as predicted from the nucleotide sequence of the full-length cDNA encoding acid β-glucosidase

Sustained high plasma mannose less sensitive to fluctuating blood glucose in glycogen storage disease type Ia children

NARCIS (Netherlands)

Nagasaka, Hironori; Yorifuji, Tohru; Bandsma, Robert H. J.; Takatani, Tomozumi; Asano, Hisaki; Mochizuki, Hiroshi; Takuwa, Mayuko; Tsukahara, Hirokazu; Inui, Ayano; Tsunoda, Tomoyuki; Komatsu, Haruki; Hiejima, Eitaro; Fujisawa, Tomoo; Hirano, Ken-ichi; Miida, Takashi; Ohtake, Akira; Taguchi, Tadao; Miwa, Ichitomo

Plasma mannose is suggested to be largely generated from liver glycogen-oriented glucose-6-phosphate. This study examined plasma mannose in glycogen storage disease type Ia (GSD Ia) lacking conversion of glucose-6-phosphate to glucose in the liver. We initially examined fasting-and postprandial 2

New insights into sulfur amino acids function in gut health and disease

Science.gov (United States)

The gastrointestinal tract (GIT) is a metabolically significant site of sulfur amino acids (SAAs) metabolism in the body. Aside from their role in protein synthesis, methionine and cysteine are involved in many biological functions and diseases. Methionine (MET) is an indispensable amino acid and is...

Survival of Escherichia coli O157:H7 in synthetic gastric fluid after cold and acid habituation in apple juice or trypticase soy broth acidified with hydrochloric acid or organic acids.

Science.gov (United States)

Uljas, H E; Ingham, S C

1998-08-01

Extreme acid tolerance of Escherichia coli O157:H7 has raised doubts about the safety of acidic foods. This study examined whether prior storage in acidic and/or cold conditions enhanced survival of E. coli O157:H7 in synthetic gastric fluid (SGF). Three E. coli O157:H7 strains were stored in trypticase soy broth (TSB; acidified with HCl, malic acid, citric acid, or lactic acid) or pH 3.5 and 6.5 (nonacidic control) apple juice at 4 and 21 degrees C for acids, suggesting that juice constituents other than organic acids protect E. coli O157:H7. Refrigeration combined with low pH best protected cells in apple juice and acidified TSB, but, compared to the nonacidic control, only acidified TSB enhanced subsequent survival in pH 2.5 SGF. Equal survival in SGF occurred after storage in pH 3.5 or 6.5 apple juice at 4 degrees C, suggesting that low temperature alone in apple juice enhanced acid tolerance. Two strains stored at 4 degrees C in TSB containing malic or citric acid subsequently survived better in SGF than cells stored in nonacidified TSB but poorer than cells stored in the presence of HCl. These differences reflect the higher pKa of these organic acids. However, subsequent survival of these strains in SGF was poorer after refrigerated storage in apple juice than in TSB containing citric or malic acids. Cells stored in lactic acid were most likely to be completely eliminated upon transfer to SGF. Differences in survival in storage media or SGF related to strain, storage conditions, or acidifier were consistent and often statistically significant (P acidic beverages may not be affected by the type of acidifier used, the subsequent survival in SGF of this pathogen may be critically dependent on this factor.

Effect of Biopreservatives on Storage Life of Papaya (Carica papaya L.

Directory of Open Access Journals (Sweden)

Fatema H. Brishti

2013-04-01

Full Text Available Normal 0 false false false EN-US X-NONE X-NONE In this experiment the effect on post-harvest preservation of papaya (Carica papaya L. fruit coated with either Aloe gel (AG; 100% or papaya leaf extract with Aloe gel (PLEAG; 1:1 was studied. To evaluate the role of coating on ripening behavior and quality of papaya the uncoated and coated fruits were stored and ripened at room temperature (25 °C-29 °C and 82-84% relative humidity. Physico-chemical properties were analyzed at 4 day intervals during the storage period. The incidence of disease attack was also visually observed. The overall results showed the superiority of AG and PLEAG coating in lengthening the shelf-life of papaya fruit compared to controls which showed significant decay from 6th day onward and complete decay within 12 days of storage. The AG and PLEAG coated fruits maintained their shelf life for 12 days and decayed at 16th day. The coated fruits also maintained their color, flavor and firmness up to 12 days of storage. An increase in ascorbic acid content (120.2 mg/100 g was also found in coated fruits in contrast to the control (59 mg/100 g. Only 27% disease incidence was observed in AG and 13% in PLEAG coated fruits as compared to control (100% during the storage period. The results of this study show that both AG and PLEAG coatings have excellent potential to be used on fresh produce to maintain quality and extend shelf-life.

Filter Paper-based Nucleic Acid Storage in High-throughput Solid Tumor Genotyping.

Science.gov (United States)

Stachler, Matthew; Jia, Yonghui; Sharaf, Nematullah; Wade, Jacqueline; Longtine, Janina; Garcia, Elizabeth; Sholl, Lynette M

2015-01-01

Molecular testing of tumors from formalin-fixed paraffin-embedded (FFPE) tissue blocks is central to clinical practice; however, it requires histology support and increases test turnaround time. Prospective fresh frozen tissue collection requires special handling, additional storage space, and may not be feasible for small specimens. Filter paper-based collection of tumor DNA reduces the need for histology support, requires little storage space, and preserves high-quality nucleic acid. We investigated the performance of tumor smears on filter paper in solid tumor genotyping, as compared with paired FFPE samples. Whatman FTA Micro Card (FTA preps) smears were prepared from 21 fresh tumor samples. A corresponding cytology smear was used to assess tumor cellularity and necrosis. DNA was isolated from FTA preps and FFPE core samples using automated methods and quantified using SYBR green dsDNA detection. Samples were genotyped for 471 mutations on a mass spectrophotometry-based platform (Sequenom). DNA concentrations from FTA preps and FFPE correlated for untreated carcinomas but not for mesenchymal tumors (Spearman σ=0.39 and σ=-0.1, respectively). Average DNA concentrations were lower from FTA preps as compared with FFPE, but DNA quality was higher with less fragmentation. Seventy-six percent of FTA preps and 86% of FFPE samples generated adequate DNA for genotyping. FTA preps tended to perform poorly for collection of DNA from pretreated carcinomas and mesenchymal neoplasms. Of the 16 paired DNA samples that were genotyped, 15 (94%) gave entirely concordant results. Filter paper-based sample preservation is a feasible alternative to FFPE for use in automated, high-throughput genotyping of carcinomas.

Wet storage integrity update

International Nuclear Information System (INIS)

Bailey, W.J.; Johnson, A.B. Jr.

1983-09-01

This report includes information from various studies performed under the Wet Storage Task of the Spent Fuel Integrity Project of the Commercial Spent Fuel Management (CSFM) Program at Pacific Northwest Laboratory. An overview of recent developments in the technology of wet storage of spent water reactor fuel is presented. Licensee Event Reports pertaining to spent fuel pools and the associated performance of spent fuel and storage components during wet storage are discussed. The current status of fuel that was examined under the CSFM Program is described. Assessments of the effect of boric acid in spent fuel pool water on the corrosion and stress corrosion cracking of stainless steel and the stress corrosion cracking of stainless steel piping containing stagnant water at spent fuel pools are discussed. A list of pertinent publications is included. 84 references, 21 figures, 11 tables

Quality evaluation of polypropylene packaged corn yogurt during storage

Science.gov (United States)

Aini, Nur; Prihananto, V.; Sustriawan, B.; Astuti, Y.; Maulina, M. R.

2018-01-01

Packaging is an important factor to control the process of quality decrease of any food product, including to determine the shelf life. The objective of this study was to determine changes quality of corn yogurt packaged using polypropylene. The method were using was package yogurt polypropylene, then it was stored in a refrigerator at 5, 10, or 15°C during 21 days. The yogurt was analysed every 7 days over a 21-day period. The results indicate that protein content decreased during storage, while the lactic acid bacteria, total acid, pH, viscosity, and total solids were increased. At the end of storage, the amount of lactic acid bacteria still fulfil the minimum requirements of a probiotic food, with a count of 6.407 log CFU/g. Overal scoring by panelist (scores ranged from 0 to 5) have a 4.78 at the beginning of storage. By the 21st day of storage, yogurt was packaging using transparent polypropylene having a score of 3.85, and that stored in opaque white packaging having a value of 3.95.

Niemann-Pick disease

Science.gov (United States)

NPD; Sphingomyelinase deficiency; Lipid storage disorder - Niemann-Pick disease; Lysosomal storage disease - Niemann-Pick ... lipofuscinoses or Batten disease (Wolman disease, cholesteryl ... metabolism of lipids. In: Kliegman RM, Stanton BF, St. Geme JW, ...

Effect of irradiation and storage on physico-chemical characteristics of garlic

International Nuclear Information System (INIS)

Wahid, M.; Mazullah; Khan, S.; Shah, A.H.

1990-01-01

Garlic bulbs (cv. G.S.I.) were irradiated at 0.05, 0.10, 0.15 or 0.20 kGy using 60 Co and stored in wooden crates for 4 months at ambient temperature (20-37°C, 50-80% RH) or low temperature (10°, 80-90% RH). Samples were monitored for sprouting, rot and weight loss. Consumer acceptability and acid value were determined throughout storage. Sprouting was apparent in control (non-irradiated) samples after 4 and 10 weeks' storage at low and ambient temperature, respectively, and reached 100 and 69% after 16 weeks' storage at low and ambient temperature, respectively. Irradiation inhibited sprouting at both temperatures. Percentage decay [unspecified] was lower in irradiated samples than in controls, which showed 31.7% and 52.9% decay, respectively, after 16 weeks' storage at ambient and low temperature. Weight loss increased from 0.2 to 28.3% and from 0.3 to 31.5% in control samples at low and ambient temperature, respectively, between 2 and 16 weeks' storage. Weight loss was less in irradiated samples. Sensory tests showed that irradiated samples retained colour, texture and odour compared with controls. There was a gradual and significant increase (from 0.448 to 2.246 mg KOH/100 g edible portion of sample) in the acid value of controls during storage. Lower doses of irradiation and storage temperature had an irregular effect on the acid value but the highest dose increased the acid value. It was concluded that irradiation had no deleterious effects on the storage properties of garlic and that a dose of 0.05 kGy could be considered cost-effective. (author)

Beneficial effect(s) of n-3 fatty acids in cardiovascular diseases: but, why and how?

Science.gov (United States)

Das, U N

2000-12-01

Low rates of coronary heart disease was found in Greenland Eskimos and Japanese who are exposed to a diet rich in fish oil. Suggested mechanisms for this cardio-protective effect focused on the effects of n-3 fatty acids on eicosanoid metabolism, inflammation, beta oxidation, endothelial dysfunction, cytokine growth factors, and gene expression of adhesion molecules; But, none of these mechanisms could adequately explain the beneficial actions of n-3 fatty acids. One attractive suggestion is a direct cardiac effect of n-3 fatty acids on arrhythmogenesis. N-3 fatty acids can modify Na+ channels by directly binding to the channel proteins and thus, prevent ischemia-induced ventricular fibrillation and sudden cardiac death. Though this is an attractive explanation, there could be other actions as well. N-3 fatty acids can inhibit the synthesis and release of pro-inflammatory cytokines such as tumor necrosis factoralpha (TNFalpha) and interleukin-1 (IL-1) and IL-2 that are released during the early course of ischemic heart disease. These cytokines decrease myocardial contractility and induce myocardial damage, enhance the production of free radicals, which can also suppress myocardial function. Further, n-3 fatty acids can increase parasympathetic tone leading to an increase in heart rate variability and thus, protect the myocardium against ventricular arrhythmias. Increased parasympathetic tone and acetylcholine, the principle vagal neurotransmitter, significantly attenuate the release of TNF, IL-1beta, IL-6 and IL-18. Exercise enhances parasympathetic tone, and the production of anti-inflammatory cytokine IL-10 which may explain the beneficial action of exercise in the prevention of cardiovascular diseases and diabetes mellitus. TNFalpha has neurotoxic actions, where as n-3 fatty acids are potent neuroprotectors and brain is rich in these fatty acids. Based on this, it is suggested that the principle mechanism of cardioprotective and neuroprotective action(s) of n-3

Evaluation of food grade solvents for lipid extraction and impact of storage temperature on fatty acid composition of edible seaweeds Laminaria digitata (Phaeophyceae) and Palmaria palmata (Rhodophyta).

Science.gov (United States)

Schmid, Matthias; Guihéneuf, Freddy; Stengel, Dagmar B

2016-10-01

This study evaluated the impact of different food- and non-food grade extraction solvents on yield and fatty acid composition of the lipid extracts of two seaweed species (Palmaria palmata and Laminaria digitata). The application of chloroform/methanol and three different food grade solvents (ethanol, hexane, ethanol/hexane) revealed significant differences in both, extraction yield and fatty acid composition. The extraction efficiency, in terms of yields of total fatty acids (TFA), was in the order: chloroform/methanol>ethanol>hexane>ethanol/hexane for both species. Highest levels of polyunsaturated fatty acids (PUFA) were achieved by the extraction with ethanol. Additionally the effect of storage temperature on the stability of PUFA in ground and freeze-dried seaweed biomass was investigated. Seaweed samples were stored for a total duration of 22months at three different temperatures (-20°C, 4°C and 20°C). Levels of TFA and PUFA were only stable after storage at -20°C for the two seaweed species. Copyright © 2016 Elsevier Ltd. All rights reserved.

Self-assembly of caseinomacropeptide as a potential key mechanism in the formation of visible storage induced aggregates in acidic whey protein isolate dispersions

DEFF Research Database (Denmark)

Villumsen, Nanna Stengaard; Jensen, Hanne Bak; Thu Le, Thao Thi

2015-01-01

Visible aggregates formed during storage in acidic whey protein isolate (WPI) dispersions represent a challenge to the beverage industry. Batch-to-batch variations are observed that prevents consistent quality and shelf-life prediction. Heat-treatment of WPI dispersions at 120°C for 20s instead...

Nucleic acid-based diagnostics for infectious diseases in public health affairs.

Science.gov (United States)

Yu, Albert Cheung-Hoi; Vatcher, Greg; Yue, Xin; Dong, Yan; Li, Mao Hua; Tam, Patrick H K; Tsang, Parker Y L; Wong, April K Y; Hui, Michael H K; Yang, Bin; Tang, Hao; Lau, Lok-Ting

2012-06-01

Infectious diseases, mostly caused by bacteria and viruses but also a result of fungal and parasitic infection, have been one of the most important public health concerns throughout human history. The first step in combating these pathogens is to get a timely and accurate diagnosis at an affordable cost. Many kinds of diagnostics have been developed, such as pathogen culture, biochemical tests and serological tests, to help detect and fight against the causative agents of diseases. However, these diagnostic tests are generally unsatisfactory because they are not particularly sensitive and specific and are unable to deliver speedy results. Nucleic acid-based diagnostics, detecting pathogens through the identification of their genomic sequences, have shown promise to overcome the above limitations and become more widely adopted in clinical tests. Here we review some of the most popular nucleic acid-based diagnostics and focus on their adaptability and applicability to routine clinical usage. We also compare and contrast the characteristics of different types of nucleic acid-based diagnostics.

Folic acid, neurodegenerative and neuropsychiatric disease.

Science.gov (United States)

Kronenberg, Golo; Colla, Michael; Endres, Matthias

2009-04-01

Folic acid plays an important role in neuroplasticity and in the maintenance of neuronal integrity. Folate is a co-factor in one-carbon metabolism during which it promotes the regeneration of methionine from homocysteine, a highly reactive sulfur-containing amino acid. Methionine may then be converted to S-adenosylmethionine (SAM), the principal methyl donor in most biosynthetic methylation reactions. On the cellular level, folate deficiency and hyperhomocysteinemia exert multiple detrimental effects. These include induction of DNA damage, uracil misincorporation into DNA and altered patterns of DNA methylation. Low folate status and elevated homocysteine increase the generation of reactive oxygen species and contribute to excitotoxicity and mitochondrial dysfunction which may lead to apoptosis. Strong epidemiological and experimental evidence links derangements of one-carbon metabolism to vascular, neurodegenerative and neuropsychiatric disease, including most prominently cerebral ischemia, Alzheimer's dementia and depression. Although firm evidence from controlled clinical trials is largely lacking, B-vitamin supplementation and homocysteine reduction may have a role especially in the primary prevention of stroke and dementia as well as as an adjunct to antidepressant pharmacotherapy.

Effect of changes in fat availability on exercise capacity in McArdle disease

DEFF Research Database (Denmark)

Andersen, S.T.; Jeppesen, T.D.; Taivassalo, T.

2009-01-01

for oxidation during exercise. DESIGN: Randomized, placebo-controlled, crossover trial. SETTING: Hospitalized care. PATIENTs: Ten patients (8 men and 2 women) with McArdle disease. INTERVENTIONS: Patients cycled at a constant workload corresponding to 70% of their maximum oxygen consumption. In random order......Ardle disease, but maximal rates of fat oxidation seem limited and cannot be increased above physiologically normal rates during exercise. This limitation is probably caused by a metabolic bottleneck in the tricarboxylic acid cycle due to impaired glycolytic flux in McArdle disease. Therapies aimed at enhancing......BACKGROUND: The major fuel for exercising muscle at low exercise intensities is fat. OBJECTIVE: To investigate the role of fat metabolism in McArdle disease (also known as glycogen storage disease type V), an inborn error of muscle glycogenolysis, by manipulating free fatty acid availability...

Chitotriosidase activity as additional biomarker in the diagnosis of lysosomal storage diseases

Directory of Open Access Journals (Sweden)

N. V. Olkhovych

2016-02-01

Full Text Available To date, several genetic variants that lead to a deficiency of chitotriosidase activity have been described. The duplication of 24 bp (dup24bp in exon 10 of the CHIT1 gene, which causes a complete loss of enzymatic activity of the gene product, is the most common among the European population. The aim of the study was to evaluate the possibility of using chitotriosidase activity as an additional biomarker in diagnosis of lysosomal storage diseases (LSDs in Ukraine, to determine this parameter in blood plasma of the patients with various lysosomal diseases and to assess the effect of the presence of dup24bp in the CHIT1 gene on this parameter. It has been shown that chitotriosidase activity in blood plasma is a convenient additional biochemical marker in the diagnosis of some LSDs, namely Gaucher disease, Niemann-Pick disease A, B, C and GM1-gangliosidosis. Reference ranges of the normal chitotriosidase activity were determined in blood plasma of Ukrainian population and found to be 8.0-53.1 nmol 4-methylumbelliferone/h·ml of plasma. The total allele frequency of the dup24bp in the CHIT1 gene in Ukrainian population was determined, which amounted to 0.26 (323/1244 that is higher than in European population. It was indicated that molecular-genetic screening of dup24bp in the CHIT1 gene is a necessary stage in a protocol for the laboratory diagnosis of Gaucher disease, Niemann-Pick disease A, B, C as well as GM1-gangliosidosis to avoid incorrect diagnosis.

Ursodeoxycholic acid in cholestatic liver disease: mechanisms of action and therapeutic use revisited

NARCIS (Netherlands)

Paumgartner, Gustav; Beuers, Ulrich

2002-01-01

Ursodeoxycholic acid (UCDA) is increasingly used for the treatment of cholestatic liver diseases. Experimental evidence suggests three major mechanisms of action: (1) protection of cholangiocytes against cytotoxicity of hydrophobic bile acids, resulting from modulation of the composition of mixed

Storage Time and Urine Biomarker Levels in the ASSESS-AKI Study.

Directory of Open Access Journals (Sweden)

Kathleen D Liu

Full Text Available Although stored urine samples are often used in biomarker studies focused on acute and chronic kidney disease, how storage time impacts biomarker levels is not well understood.866 subjects enrolled in the NIDDK-sponsored ASsessment, Serial Evaluation, and Subsequent Sequelae in Acute Kidney Injury (ASSESS-AKI Study were included. Samples were processed under standard conditions and stored at -70°C until analyzed. Kidney injury molecule-1 (KIM-1, neutrophil gelatinase-associated lipocalin (NGAL, interleukin-18 (IL-18, and liver fatty acid binding protein (L-FABP were measured in urine samples collected during the index hospitalization or an outpatient visit 3 months later. Mixed effects models were used to determine the effect of storage time on biomarker levels and stratified by visit.Median storage was 17.8 months (25-75% IQR 10.6-23.7 for samples from the index hospitalization and 14.6 months (IQR 7.3-20.4 for outpatient samples. In the mixed effects models, the only significant association between storage time and biomarker concentration was for KIM-1 in outpatient samples, where each month of storage was associated with a 1.7% decrease (95% CI -3% to -0.3%. There was no relationship between storage time and KIM-1 levels in samples from the index hospitalization.There was no significant impact of storage time over a median of 18 months on urine KIM-1, NGAL, IL-18 or L-FABP in hospitalized samples; a statistically significant effect towards a decrease over time was noted for KIM-1 in outpatient samples. Additional studies are needed to determine whether longer periods of storage at -70°C systematically impact levels of these analytes.

Fracture Dissolution of Carbonate Rock: An Innovative Process for Gas Storage

Energy Technology Data Exchange (ETDEWEB)

James W. Castle; Ronald W. Falta; David Bruce; Larry Murdoch; Scott E. Brame; Donald Brooks

2006-10-31

The goal of the project is to develop and assess the feasibility and economic viability of an innovative concept that may lead to commercialization of new gas-storage capacity near major markets. The investigation involves a new approach to developing underground gas storage in carbonate rock, which is present near major markets in many areas of the United States. Because of the lack of conventional gas storage and the projected growth in demand for storage capacity, many of these areas are likely to experience shortfalls in gas deliverability. Since depleted gas reservoirs and salt formations are nearly non-existent in many areas, alternatives to conventional methods of gas storage are required. The need for improved methods of gas storage, particularly for ways to meet peak demand, is increasing. Gas-market conditions are driving the need for higher deliverability and more flexibility in injection/withdrawal cycling. In order to meet these needs, the project involves an innovative approach to developing underground storage capacity by creating caverns in carbonate rock formations by acid dissolution. The basic concept of the acid-dissolution method is to drill to depth, fracture the carbonate rock layer as needed, and then create a cavern using an aqueous acid to dissolve the carbonate rock. Assessing feasibility of the acid-dissolution method included a regional geologic investigation. Data were compiled and analyzed from carbonate formations in six states: Indiana, Ohio, Kentucky, West Virginia, Pennsylvania, and New York. To analyze the requirements for creating storage volume, the following aspects of the dissolution process were examined: weight and volume of rock to be dissolved; gas storage pressure, temperature, and volume at depth; rock solubility; and acid costs. Hydrochloric acid was determined to be the best acid to use because of low cost, high acid solubility, fast reaction rates with carbonate rock, and highly soluble products (calcium chloride

Effects of Starter Culture and Storage Temperature on Functional, Microbial and Sensory Characteristics of Kefir during Storage

Directory of Open Access Journals (Sweden)

Tayyebeh Sarlak

2017-03-01

Full Text Available The aim of this study was to investigate the effects of starters and storage temperature (4 ºC, 25 ºC on microbiological and physicochemical properties, volatile compounds and sensory evaluation of kefir. Kefirs produced by KFA and Chr. Hansen starters were stored at 4 ºC and 25 ºC for 40 days. pH and acidity at 4 ºC did not change (p ≥ 0.05, while at 25 ºC pH and total solid decreased as well as acidity. Concentrations of acetaldehyde and ethanol increased (p < 0.05. No significant differences (p ≥ 0.05 were observed in protein and non-protein-nitrogen for both samples. Sensory evaluation revealed that storage did not affect texture and color of samples at 4 ºC. Kefir produced by Chr. Hansen starter stored at 4 ºC had the highest acceptability until 40-day storage and was preferred by the panelists.

Transcription factor EB: from master coordinator of lysosomal pathways to candidate therapeutic target in degenerative storage diseases.

Science.gov (United States)

Sardiello, Marco

2016-05-01

The lysosome is the main catabolic hub of the cell. Owing to its role in fundamental processes such as autophagy, plasma membrane repair, mTOR signaling, and maintenance of cellular homeostasis, the lysosome has a profound influence on cellular metabolism and human health. Indeed, inefficient or impaired lysosomal function has been implicated in the pathogenesis of a number of degenerative diseases affecting various organs and tissues, most notably the brain, liver, and muscle. The discovery of the coordinated lysosomal expression and regulation (CLEAR) genetic program and its master controller, transcription factor EB (TFEB), has provided an unprecedented tool to study and manipulate lysosomal function. Most lysosome-based processes-including macromolecule degradation, autophagy, lysosomal exocytosis, and proteostasis-are under the transcriptional control of TFEB. Interestingly, impaired TFEB signaling has been suggested to be a contributing factor in the pathogenesis of several degenerative storage diseases. Preclinical studies based on TFEB exogenous expression to reinstate TFEB activity or promote CLEAR network-based lysosomal enhancement have highlighted TFEB as a candidate therapeutic target for the treatment of various degenerative storage diseases. © 2016 The Authors. Annals of the New York Academy of Sciences published by Wiley Periodicals, Inc. on behalf of New York Academy of Sciences.

Biochemical and proteomic analysis of grape berries (Vitis labruscana) during cold storage upon postharvest salicylic acid treatment.

Science.gov (United States)

Cai, Han; Yuan, Xiaozhuan; Pan, Jiaojiao; Li, Huan; Wu, Ziming; Wang, Yun

2014-10-15

Salicylic acid (SA) treatment has been widely used to maintain fruit quality during postharvest storage. To elucidate the molecular mechanism related to this treatment, the effect of SA treatment on fruit quality as well as protein expression profiles of grape berries (Vitis labruscana cv. Kyoho) during the subsequent cold storage was evaluated. As expected, SA treatment inhibited postharvest loss and chilling damage by reducing fruit softening and membrane damage and slowing weight loss. A gel-based proteomic approach was designed to screen for differentially expressed proteins in SA-treated and control grape berries. A total of 69 differentially accumulated proteins were successfully identified by matrix-assisted laser desorption/ionization time-of-flight (MALDI-TOF) mass spectrometry, which can be functionally classified into eight categories. Among these proteins, antioxidant enzymes including ascorbate peroxidase, oxidoreductase, and glutathione S-transferase were induced, and the abundances of several defense-related proteins, such as heat shock protein (HSP) and temperature-induced lipocalin, were up-regulated by SA treatment. In addition, proteins involved in carbohydrate catabolism and energy production were also induced by SA treatment. Interpretation of the data for differential accumulation of proteins revealed that the effect of SA on reducing postharvest losses and chilling damage of grape berries during cold storage may be due to activated defense responses and carbohydrate metabolism and higher levels of energy status.

Influence of different storage times and temperatures on blood gas and acid-base balance in ovine venous blood

Directory of Open Access Journals (Sweden)

H.A. Hussein

2013-01-01

Full Text Available The present study was designed to investigate the effects of storage temperature and time on blood gas and acid-base balance of ovine venous blood. Ten clinically healthy sheep were used in this study. A total number of 30 blood samples, were divided into three different groups, and were stored in a refrigerator adjusted to +4 ºC (Group I, n = 10, at RT of about 22-25 ºC (Group II, n = 10 and in an incubator adjusted to 37 ºC (Group III, n = 10 for up to 48 h. Blood samples were analysed for blood gas and acid-base indices at 0, 1, 2, 3, 4, 5, 6, 12, 24 and 48 h of storage. In comparison to the baseline value (0, there were significant decreases of blood pH of samples stored at RT and in the incubator after 1 h (p<0.05, the pH value of refrigerated blood samples exhibited insignificant changes during the study (p<0.05. Mean values of pCO2 showed a significant increase in Group I and Group III after 1 h then a progressive decrease after 12 h in all Groups. Mean pO2 values were significantly higher for Group I after 2 h and for Groups II and III after 1 h (p<0.05. In general, base excess decreased significantly for all the groups during the study especially in Groups II and III. In comparison with baseline values, in all groups, bicarbonate (HCO3 increased between 1 h and 6 h (p<0.05, and later decreased at the end of the study (p<0.05. In conclusion, status of acid-base indices of the samples stored at refrigerator and RT were found within normal reference range and it may be of clinical diagnostic use for up to 6 h.

Novel retinoic acid receptor alpha agonists for treatment of kidney disease.

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Yifei Zhong

Full Text Available Development of pharmacologic agents that protect podocytes from injury is a critical strategy for the treatment of kidney glomerular diseases. Retinoic acid reduces proteinuria and glomerulosclerosis in multiple animal models of kidney diseases. However, clinical studies are limited because of significant side effects of retinoic acid. Animal studies suggest that all trans retinoic acid (ATRA attenuates proteinuria by protecting podocytes from injury. The physiological actions of ATRA are mediated by binding to all three isoforms of the nuclear retinoic acid receptors (RARs: RARα, RARβ, and RARγ. We have previously shown that ATRA exerts its renal protective effects mainly through the agonism of RARα. Here, we designed and synthesized a novel boron-containing derivative of the RARα-specific agonist Am580. This new derivative, BD4, binds to RARα receptor specifically and is predicted to have less toxicity based on its structure. We confirmed experimentally that BD4 binds to RARα with a higher affinity and exhibits less cellular toxicity than Am580 and ATRA. BD4 induces the expression of podocyte differentiation markers (synaptopodin, nephrin, and WT-1 in cultured podocytes. Finally, we confirmed that BD4 reduces proteinuria and improves kidney injury in HIV-1 transgenic mice, a model for HIV-associated nephropathy (HIVAN. Mice treated with BD4 did not develop any obvious toxicity or side effect. Our data suggest that BD4 is a novel RARα agonist, which could be used as a potential therapy for patients with kidney disease such as HIVAN.

Polyunsaturated fatty acids and their metabolites in brain function and disease.

Science.gov (United States)

Bazinet, Richard P; Layé, Sophie

2014-12-01

The brain is highly enriched with fatty acids. These include the polyunsaturated fatty acids (PUFAs) arachidonic acid and docosahexaenoic acid, which are largely esterified to the phospholipid cell membrane. Once PUFAs are released from the membrane, they can participate in signal transduction, either directly or after enzymatic conversion to a variety of bioactive derivatives ('mediators'). PUFAs and their mediators regulate several processes within the brain, such as neurotransmission, cell survival and neuroinflammation, and thereby mood and cognition. PUFA levels and the signalling pathways that they regulate are altered in various neurological disorders, including Alzheimer's disease and major depression. Diet and drugs targeting PUFAs may lead to novel therapeutic approaches for the prevention and treatment of brain disorders.

Neutral lipid-storage disease with myopathy and extended phenotype with novel PNPLA2 mutation.

Science.gov (United States)

Massa, Roberto; Pozzessere, Simone; Rastelli, Emanuele; Serra, Laura; Terracciano, Chiara; Gibellini, Manuela; Bozzali, Marco; Arca, Marcello

2016-04-01

Neutral lipid-storage disease with myopathy is caused by mutations in PNPLA2, which produce skeletal and cardiac myopathy. We report a man with multiorgan neutral lipid storage and unusual multisystem clinical involvement, including cognitive impairment. Quantitative brain MRI with voxel-based morphometry and extended neuropsychological assessment were performed. In parallel, the coding sequences and intron/exon boundaries of the PNPLA2 gene were screened by direct sequencing. Neuropsychological assessment revealed global cognitive impairment, and brain MRI showed reduced gray matter volume in the temporal lobes. Molecular characterization revealed a novel homozygous mutation in exon 5 of PNPLA2 (c.714C>A), resulting in a premature stop codon (p.Cys238*). Some PNPLA2 mutations, such as the one described here, may present with an extended phenotype, including brain involvement. In these cases, complete neuropsychological testing, combined with quantitative brain MRI, may help to characterize and quantify cognitive impairment. © 2016 Wiley Periodicals, Inc.

Mechanisms of acid reflux and how refluxed Acid extends proximally in patients with non-erosive reflux disease.

Science.gov (United States)

Sano, Hirohito; Iwakiri, Katsuhiko; Kawami, Noriyuki; Tanaka, Yuriko; Sakamoto, Choitsu

2014-01-01

The mechanisms that cause acid reflux in patients with non-erosive reflux disease (NERD), including those that determine how acid extends proximally, are not yet clear. Concurrent esophageal manometry and pH monitoring were performed for 3 h after a meal in 13 patients with NERD, 12 with mild reflux esophagitis (RE), and 13 healthy subjects (HS). Transient lower esophageal sphincter (LES) relaxation (TLESR) was the major mechanism of acid reflux in all three groups. LES pressure did not differ between the groups. At 2 cm above the LES, there were no differences between the three groups in the number of TLESR-related acid reflux episodes, rate of TLESRs and rate of acid reflux during TLESR. However, at 7 cm above the LES, the rate of acid reflux during TLESRs was significantly higher in patients with NERD (mean ± SEM 42.3 ± 4.8) than in those with mild RE (28.0 ± 3.8) and HS (10.8 ± 2.5). TLESRs are the sole motor events underlying acid reflux episodes in patients with NERD. Acid extends proximally more readily in patients with NERD than in HS and those with mild RE.

Recombinant human acid alpha-glucosidase: high level production in mouse milk, biochemical characteristics, correction of enzyme deficiency in GSDII KO mice

NARCIS (Netherlands)

A.G.A. Bijvoet (Agnes); M.A. Kroos (Marian); F.R. Pieper (Frank); M. Van der Vliet (Martin); H.A. de Boer (Herman); A.T. van der Ploeg (Ans); M.Ph. Verbeet (Martin); A.J.J. Reuser (Arnold)

1998-01-01

textabstractGlycogen storage disease type II (GSDII) is caused by lysosomal acid alpha-glucosidase deficiency. Patients have a rapidly fatal or slowly progressive impairment of muscle function. Enzyme replacement therapy is under investigation. For large-scale, cost-effective

In vivo hepatic lipid quantification using MRS at 7 Tesla in a mouse model of glycogen storage disease type 1a.

Science.gov (United States)

Ramamonjisoa, Nirilanto; Ratiney, Helene; Mutel, Elodie; Guillou, Herve; Mithieux, Gilles; Pilleul, Frank; Rajas, Fabienne; Beuf, Olivier; Cavassila, Sophie

2013-07-01

The assessment of liver lipid content and composition is needed in preclinical research to investigate steatosis and steatosis-related disorders. The purpose of this study was to quantify in vivo hepatic fatty acid content and composition using a method based on short echo time proton magnetic resonance spectroscopy (MRS) at 7 Tesla. A mouse model of glycogen storage disease type 1a with inducible liver-specific deletion of the glucose-6-phosphatase gene (L-G6pc(-/-)) mice and control mice were fed a standard diet or a high-fat/high-sucrose (HF/HS) diet for 9 months. In control mice, hepatic lipid content was found significantly higher with the HF/HS diet than with the standard diet. As expected, hepatic lipid content was already elevated in L-G6pc(-/-) mice fed a standard diet compared with control mice. L-G6pc(-/-) mice rapidly developed steatosis which was not modified by the HF/HS diet. On the standard diet, estimated amplitudes from olefinic protons were found significantly higher in L-G6pc(-/-) mice compared with that in control mice. L-G6pc(-/-) mice showed no noticeable polyunsaturation from diallylic protons. Total unsaturated fatty acid indexes measured by gas chromatography were in agreement with MRS measurements. These results showed the great potential of high magnetic field MRS to follow the diet impact and lipid alterations in mouse liver.

Sulfur amino acids in Cushing's disease: insight in homocysteine and taurine levels in patients with active and cured disease.

Science.gov (United States)

Faggiano, Antongiulio; Melis, Daniela; Alfieri, Raffaele; De Martino, MariaCristina; Filippella, Mariagiovanna; Milone, Francesco; Lombardi, Gaetano; Colao, Annamaria; Pivonello, Rosario

2005-12-01

Cushing's syndrome is associated with an increased cardiovascular risk. Although a series of cardiovascular risk factors have been identified, sulfur amino acids (SAAs), recently indicated as independent cardiovascular risk factors, have been poorly investigated in patients with Cushing's syndrome. The aim of this cross-sectional controlled study was to evaluate serum and urinary levels and urinary excretion rate (ER) of SAAs in patients with Cushing's disease (CD) during the active disease and after long-term disease remission. Forty patients with CD (20 with active disease and 20 with cured disease for at least 5 yr) and 40 controls entered the study. Serum and urinary concentrations and urinary ER of SAAs, namely methionine, cystine, homocysteine, and taurine, were measured by means of cationic exchange HPLC. Serum folic acid and vitamin B12 levels were also evaluated in patients and controls and correlated to SAA levels. CD patients with active disease had higher serum and urinary concentrations of cystine and homocysteine, and lower serum and higher urinary concentrations and ER of taurine than cured patients and controls. Vitamin B12 levels were significantly decreased in patients with active disease compared with cured patients and controls, whereas folic acid levels were slightly decreased in patients than in controls. In patients with active CD, urinary cortisol concentrations were significantly and inversely correlated to serum taurine and directly correlated to taurine urinary ER, and fasting serum glucose levels were significantly correlated to taurine urinary ER. At the multiple regression analysis, urinary cortisol concentrations were the best predictors of taurine ER. CD is associated with hyperhomocysteinemia and hypotaurinemia. Glucocorticoid excess, acting directly or indirectly, seems to be the most responsible for this imbalance in SAA levels. The long-term disease remission is accompanied by normalization of SAA levels. Hyperhomocysteinemia and

Second International Conference on Batteries for Utility Energy Storage

Energy Technology Data Exchange (ETDEWEB)

NONE

1989-07-24

This is a collection of essays presented at the above-named conference held at New Port Beach, U.S., from July 24 through 28, 1989. At the utility energy storage session, it is found that the 100kW-capable Na-S battery system of the Kansai Electric Power Company, Inc., works effectively in levelling peakloads at storage efficiency of 70%. A Chino lead-acid battery system is also described. A lead-acid battery system of the BEWAG Corporation of Germany equipped with tubular electrodes is described. For application by the consuming party, system behavior relative to duty cycle control, sudden request for energy storage, power factor, and load adjustment is discussed. Use of a valve-controlled lead-acid battery is introduced, which is to be used as a stand-by system (such as an uninterruptible power supply) or for certain types of cyclic duties. At the 4th session, economic and technical models are exhibited. Computer-aided peakload prediction, battery storage system technology, economic parameters, profitability, etc., are explained for use by the consuming party in a peakload shaving battery system. The Zn/Br battery, redox-flow battery, and other advanced technologies are also presented. (NEDO)

Omega-3 polyunsaturated fatty acid (fish oil) supplementation and the prevention of clinical cardiovascular disease

Science.gov (United States)

Multiple randomized controlled trials (RCTs) have assessed the effects of supplementation with eicosapentaenoic acid plus docosahexaenoic acid (omega-3 polyunsaturated fatty acids, commonly called fish oils) on the occurrence of clinical cardiovascular diseases. Although the effects of supplementati...

Plasma phospholipid fatty acid concentration and incident coronary heart disease in men and women: the EPIC-Norfolk prospective study.

Directory of Open Access Journals (Sweden)

Kay-Tee Khaw

Full Text Available The lack of association found in several cohort studies between dietary saturated fat and coronary heart disease (CHD risk has renewed debate over the link between dietary fats and CHD.We assessed the relationship between plasma phospholipid fatty acid (PFA concentration and incident CHD using a nested case control design within a prospective study (EPIC-Norfolk of 25,639 individuals aged 40-79 years examined in 1993-1997 and followed up to 2009. Plasma PFA concentrations were measured by gas chromatography in baseline samples retrieved from frozen storage. In 2,424 men and women with incident CHD compared with 4,930 controls alive and free of cardiovascular disease, mean follow-up 13 years, saturated PFA (14:0, 16:0,18:0 plasma concentrations were significantly associated with increased CHD risk (odds ratio [OR] 1.75, 95% CI 1.27-2.41, p<0.0001, in top compared to bottom quartiles (Q, and omega-6 polyunsaturated PFA concentrations were inversely related (OR 0.77, 0.60-0.99, p<0.05 after adjusting for age, sex, body mass index, blood pressure, smoking, alcohol intake, plasma vitamin C, social class, education, and other PFAs. Monounsaturated PFA, omega-3 PFA, and trans PFA concentrations were not significantly associated with CHD. Odd chain PFA (15:0, 17:0 concentrations were significantly inversely associated with CHD (OR 0.73, 0.59-0.91, p<0.001, Q4 versus Q1. Within families of saturated PFA or polyunsaturated PFA, significantly heterogeneous relationships with CHD were observed for individual fatty acids.In this study, plasma concentrations of even chain saturated PFA were found to be positively and omega-6 polyunsaturated PFA inversely related to subsequent coronary heart disease risk. These findings are consistent with accumulating evidence suggesting a protective role of omega-6 fats substituting for saturated fats for CHD prevention.

Effects of a propionic acid-based preservative on storage characteristics, nutritive value, and energy content for alfalfa hays packaged in large, round bales

Science.gov (United States)

During 2009 and 2010, alfalfa (Medicago sativa L.) hays from two cuttings that were harvested from the same field site were used to evaluate the effects of a propionic acid-based preservative on the storage characteristics and nutritive value of hays stored as large, round bales. A total of 87 large...

Increased lipogenesis and resistance of lipoproteins to oxidative modification in two patients with glycogen storage disease type 1a

NARCIS (Netherlands)

Bandsma, RHJ; Rake, JP; Visser, G; Neese, RA; Hellerstein, MK; van Duyvenvoorde, W; Princen, HMG; Stellaard, F; Smit, GPA; Kuipers, F

We describe 2 patients with glycogen storage disease type la and severe hyperlipidemia without premature atherosclerosis. Susceptibility of low-density lipoproteins to oxidation was decreased, possibly related to the similar to40-fold increase in palmitate synthesis altering lipoprotein saturated

ALGLUCOSIDASE ALFA — A NEW STAGE IN THE THERAPY OF INFANTILE POMPE DISEASE

Directory of Open Access Journals (Sweden)

E. N. Basargina

2014-01-01

Full Text Available Pompe disease is a rare severe hereditary disease caused by excessive glycogen storage in organs and target tissues due to the acid α-glucosidase gene mutation. Infantile and adult Pompe disease is characterized by involvement of cardiovascular, respiratory and muscular systems in the pathological process. The only specific method of treating Pompe disease is enzyme replacement therapy (intravenous administration of recombinant human acid glucosidase, the effectiveness whereof depends on the time the therapy started. Since such a therapy was introduced into practice, Pompe disease mortality decreased by 79%. 6 children with infantile Pompe disease were observed and treated at the cardiovascular care unit of the Scientific Center of Children’s Health in 2011‑2014. The article presents a clinical case demonstrating capabilities of diagnosing infantile Pompe disease in Russia and effective application of alglucosidase alfa in 4-month-old child. 

Effect of gamma-irradiation and extended storage on chemical quality in onion (Allium cepa L.)

International Nuclear Information System (INIS)

Croci, C.A.; Banek, S.A.; Curzio, O.A.

1995-01-01

The effects of gamma-irradiation and long-term storage on the chemical quality of the Valenciana sintética 14 onion variety were determined under warehouse conditions in two sets of bulbs grown consecutively in 1988 and 1989. In both years irradiated and non-irradiated bulbs showed similar behaviour in terms of carbohydrate and ascorbic acid contents throughout the 300 days of storage. It was found that the carbohydrate content significantly decreased in irradiated and non-irradiated samples up to 180 days of storage. The storage time was found not to have a significant effect on the ascorbic acid content of bulbs. The carbohydrate and ascorbic acid contents were found to be higher in the irradiated and non-irradiated bulbs grown in 1988. Neither storage time nor gamma-irradiation nor the specific year significantly affected dry matter or acidity. Gamma-irradiation did not significantly affect flavour strength in terms of total pyruvate content

The antioxidant system of seminal fluid during in vitro storage of sterlet Acipenser ruthenus sperm.

Science.gov (United States)

Dzyuba, Viktoriya; Cosson, Jacky; Dzyuba, Borys; Yamaner, Gunes; Rodina, Marek; Linhart, Otomar

2016-04-01

The role of the seminal fluid antioxidant system in protection against damage to spermatozoa during in vitro sperm storage is unclear. This study investigated the effect of in vitro storage of sterlet Acipenser ruthenus spermatozoa together with seminal fluid for 36 h at 4 °C on spermatozoon motility rate and curvilinear velocity, thiobarbituric acid reactive substance level, and components of enzyme and non-enzyme antioxidant system (superoxide dismutase and catalase activity and uric acid concentration) in seminal fluid. Spermatozoon motility parameters after sperm storage were significantly decreased, while the level of thiobarbituric acid reactive substances, activity of superoxide dismutase and catalase, and uric acid concentration did not change. Our findings suggest that the antioxidant system of sterlet seminal fluid is effective in preventing oxidative stress during short-term sperm storage and prompt future investigations of changes in spermatozoon homeostasis and in spermatozoon plasma membrane structure which are other possible reasons of spermatozoon motility deterioration upon sperm storage.

Effect of CaCl2 and controlled atmosphere storage on phytochemical attributes of Guava

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Muhammad Sameem JAVED

2017-10-01

Full Text Available Abstract Guava is very delicate and alluring fruit which is being ignored since very long time despite of highly nutritious fruit and rich source of Vitamin C. It contains Vitamin C 2-3 times more than orange. Naturally the guava fruit is enriched with vitamin C and polyphenoles. Guavas fruits after harvesting were dunked in solutions of CaCl2 (1, 2 and 3% at room temperature for 5 minutes and stored for 24 days in 5% CO2 level at temperature of 10±1°C, while the humidity level of storage chamber were 80%. The stored fruits were analyzed at 6 days of interval for sugars (glucose, fructose and sucrose g/100g total phenolic contents (mgGAE/100g, antioxidant activity (µmolTE/g, and organic acids (citric, tartaric, ascorbic, and malic acids mg/100 g. The total phenolic content and antioxidant activity of guava fruits were declined during progression in storage but in fewer amounts as compared to room storage condition. Citric acid and ascorbic acid contents were reduced with the progression in storage, however tartaric and malic acid values were amplified at end of storage but the rate of changes were slower. The pretreatments in combination with modified atmosphere storage escalate the shelf life of guava and slow down nutritional degradation process.

Ursodeoxycholic acid treatment in patients with cystic fibrosis at risk for liver disease.

Science.gov (United States)

Siano, Maria; De Gregorio, Fabiola; Boggia, Bartolo; Sepe, Angela; Ferri, Pasqualina; Buonpensiero, Paolo; Di Pasqua, Antonio; Raia, Valeria

2010-06-01

Meconium ileus has been detected as a risk factor for development of liver disease in cystic fibrosis, with influence on morbidity and mortality. To evaluate the effect of early treatment with ursodeoxycholic acid in patients with cystic fibrosis and meconium ileus to prevent chronic hepatic involvement and to explore the potential role of therapy on clinical outcomes. 26 cystic fibrosis patients with meconium ileus (16 M, mean age 8,4 years, range 3,5-9) were assigned to two groups: group 1 (14 patients) treated early with ursodeoxycholic acid (UDCAe); group 2 (12 patients) treated with ursodeoxycholic acid at the onset of cystic fibrosis liver disease (UDCAd). Anthropometric data, pulmonary function tests, pancreatic status, complications such as diabetes, hepatic involvement and Pseudomonas aeruginosa colonisation were compared among groups. A higher prevalence of cystic fibrosis chronic liver disease was observed in the UDCAd group with a statistically significant difference at 9 years of age (p<0.05). Chronic infection by P. aeruginosa was found in 7% of UDCAe and 33% of UDCAd (p<0.05). No differences were observed in nutritional status and other complications. Early treatment with ursodeoxycholic acid may be beneficial in patients at risk of developing cystic fibrosis chronic liver disease such as those with meconium ileus. Multicentre studies should be encouraged to confirm these data. Copyright 2009 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

Proteome-based systems biology analysis of the diabetic mouse aorta reveals major changes in fatty acid biosynthesis as potential hallmark in diabetes mellitus-associated vascular disease.

Science.gov (United States)

Husi, Holger; Van Agtmael, Tom; Mullen, William; Bahlmann, Ferdinand H; Schanstra, Joost P; Vlahou, Antonia; Delles, Christian; Perco, Paul; Mischak, Harald

2014-04-01

Macrovascular complications of diabetes mellitus are a major risk factor for cardiovascular morbidity and mortality. Currently, studies only partially described the molecular pathophysiology of diabetes mellitus-associated effects on vasculature. However, better understanding of systemic effects is essential in unraveling key molecular events in the vascular tissue responsible for disease onset and progression. Our overall aim was to get an all-encompassing view of diabetes mellitus-induced key molecular changes in the vasculature. An integrative proteomic and bioinformatics analysis of data from aortic vessels in the low-dose streptozotocin-induced diabetic mouse model (10 animals) was performed. We observed pronounced dysregulation of molecules involved in myogenesis, vascularization, hypertension, hypertrophy (associated with thickening of the aortic wall), and a substantial reduction of fatty acid storage. A novel finding is the pronounced downregulation of glycogen synthase kinase-3β (Gsk3β) and upregulation of molecules linked to the tricarboxylic acid cycle (eg, aspartate aminotransferase [Got2] and hydroxyacid-oxoacid transhydrogenase [Adhfe1]). In addition, pathways involving primary alcohols and amino acid breakdown are altered, potentially leading to ketone-body production. A number of these findings were validated immunohistochemically. Collectively, the data support the hypothesis that in this diabetic model, there is an overproduction of ketone-bodies within the vessels using an alternative tricarboxylic acid cycle-associated pathway, ultimately leading to the development of atherosclerosis. Streptozotocin-induced diabetes mellitus in animals leads to a reduction of fatty acid biosynthesis and an upregulation of an alternative ketone-body formation pathway. This working hypothesis could form the basis for the development of novel therapeutic intervention and disease management approaches.

Seasonal energy storage - PV-hydrogen systems

Energy Technology Data Exchange (ETDEWEB)

Leppaenen, J. [Neste Oy/NAPS (Finland)

1998-10-01

PV systems are widely used in remote areas e.g. in telecommunication systems. Typically lead acid batteries are used as energy storage. In northern locations seasonal storage is needed, which however is too expensive and difficult to realise with batteries. Therefore, a PV- battery system with a diesel backup is sometimes used. The disadvantages of this kind of system for very remote applications are the need of maintenance and the need to supply the fuel. To overcome these problems, it has been suggested to use hydrogen technologies to make a closed loop autonomous energy storage system

Effect of chemical treatments on hydrogen storage behaviors of multi-walled carbon nanotubes

International Nuclear Information System (INIS)

Lee, Seul-Yi; Park, Soo-Jin

2010-01-01

In this work, the hydrogen storage behaviors of chemically treated multi-walled carbon nanotubes (MWNTs) were investigated. The surface properties of the functionalized MWNTs were confirmed by Fourier transfer infrared spectroscopy, X-ray diffraction, the Boehm titration method, and zeta-potential measurements. The hydrogen storage capacity of the MWNTs was evaluated at 298 K and 100 bar. In the experimental results, it was found that the chemical treatments introduced functional groups onto the MWNT surfaces. The amount of hydrogen storage was enhanced, by acidic surface treatment, to 0.42 wt.% in the acidic-treated MWNTs compared with 0.26 wt.% in the as-received MWNTs. Meanwhile, the basic surface treatment actually reduced the hydrogen storage capacity, to 0.24 wt.% in the basic-treated MWNTs sample. Consequently, it could be concluded that hydrogen storage is greatly influenced by the acidic characteristics of MWNT surfaces, resulting in enhanced electron acceptor-donor interaction at interfaces.

Decreased hepatotoxic bile acid composition and altered synthesis in progressive human nonalcoholic fatty liver disease

International Nuclear Information System (INIS)

Lake, April D.; Novak, Petr; Shipkova, Petia; Aranibar, Nelly; Robertson, Donald; Reily, Michael D.; Lu, Zhenqiang; Lehman-McKeeman, Lois D.; Cherrington, Nathan J.

2013-01-01

Bile acids (BAs) have many physiological roles and exhibit both toxic and protective influences within the liver. Alterations in the BA profile may be the result of disease induced liver injury. Nonalcoholic fatty liver disease (NAFLD) is a prevalent form of chronic liver disease characterized by the pathophysiological progression from simple steatosis to nonalcoholic steatohepatitis (NASH). The hypothesis of this study is that the ‘classical’ (neutral) and ‘alternative’ (acidic) BA synthesis pathways are altered together with hepatic BA composition during progression of human NAFLD. This study employed the use of transcriptomic and metabolomic assays to study the hepatic toxicologic BA profile in progressive human NAFLD. Individual human liver samples diagnosed as normal, steatosis, and NASH were utilized in the assays. The transcriptomic analysis of 70 BA genes revealed an enrichment of downregulated BA metabolism and transcription factor/receptor genes in livers diagnosed as NASH. Increased mRNA expression of BAAT and CYP7B1 was observed in contrast to decreased CYP8B1 expression in NASH samples. The BA metabolomic profile of NASH livers exhibited an increase in taurine together with elevated levels of conjugated BA species, taurocholic acid (TCA) and taurodeoxycholic acid (TDCA). Conversely, cholic acid (CA) and glycodeoxycholic acid (GDCA) were decreased in NASH liver. These findings reveal a potential shift toward the alternative pathway of BA synthesis during NASH, mediated by increased mRNA and protein expression of CYP7B1. Overall, the transcriptomic changes of BA synthesis pathway enzymes together with altered hepatic BA composition signify an attempt by the liver to reduce hepatotoxicity during disease progression to NASH. - Highlights: ► Altered hepatic bile acid composition is observed in progressive NAFLD. ► Bile acid synthesis enzymes are transcriptionally altered in NASH livers. ► Increased levels of taurine and conjugated bile acids

Decreased hepatotoxic bile acid composition and altered synthesis in progressive human nonalcoholic fatty liver disease

Energy Technology Data Exchange (ETDEWEB)

Lake, April D. [University of Arizona, Department of Pharmacology and Toxicology, Tucson, AZ 85721 (United States); Novak, Petr [Biology Centre ASCR, Institute of Plant Molecular Biology, Ceske Budejovice 37001 (Czech Republic); Shipkova, Petia; Aranibar, Nelly; Robertson, Donald; Reily, Michael D. [Pharmaceutical Candidate Optimization, Bristol-Myers Squibb Co., Princeton, NJ 08543 (United States); Lu, Zhenqiang [The Arizona Statistical Consulting Laboratory, University of Arizona, Tucson, AZ 85721 (United States); Lehman-McKeeman, Lois D. [Pharmaceutical Candidate Optimization, Bristol-Myers Squibb Co., Princeton, NJ 08543 (United States); Cherrington, Nathan J., E-mail: cherrington@pharmacy.arizona.edu [University of Arizona, Department of Pharmacology and Toxicology, Tucson, AZ 85721 (United States)

2013-04-15

Bile acids (BAs) have many physiological roles and exhibit both toxic and protective influences within the liver. Alterations in the BA profile may be the result of disease induced liver injury. Nonalcoholic fatty liver disease (NAFLD) is a prevalent form of chronic liver disease characterized by the pathophysiological progression from simple steatosis to nonalcoholic steatohepatitis (NASH). The hypothesis of this study is that the ‘classical’ (neutral) and ‘alternative’ (acidic) BA synthesis pathways are altered together with hepatic BA composition during progression of human NAFLD. This study employed the use of transcriptomic and metabolomic assays to study the hepatic toxicologic BA profile in progressive human NAFLD. Individual human liver samples diagnosed as normal, steatosis, and NASH were utilized in the assays. The transcriptomic analysis of 70 BA genes revealed an enrichment of downregulated BA metabolism and transcription factor/receptor genes in livers diagnosed as NASH. Increased mRNA expression of BAAT and CYP7B1 was observed in contrast to decreased CYP8B1 expression in NASH samples. The BA metabolomic profile of NASH livers exhibited an increase in taurine together with elevated levels of conjugated BA species, taurocholic acid (TCA) and taurodeoxycholic acid (TDCA). Conversely, cholic acid (CA) and glycodeoxycholic acid (GDCA) were decreased in NASH liver. These findings reveal a potential shift toward the alternative pathway of BA synthesis during NASH, mediated by increased mRNA and protein expression of CYP7B1. Overall, the transcriptomic changes of BA synthesis pathway enzymes together with altered hepatic BA composition signify an attempt by the liver to reduce hepatotoxicity during disease progression to NASH. - Highlights: ► Altered hepatic bile acid composition is observed in progressive NAFLD. ► Bile acid synthesis enzymes are transcriptionally altered in NASH livers. ► Increased levels of taurine and conjugated bile acids

The role of bile acids in the pathogenesis of bowel diseases

Directory of Open Access Journals (Sweden)

Magdalena Panek-Jeziorna

2017-08-01

Full Text Available Bile acids not only play a cardinal role in the digestion and absorption of fat and fat-soluble vitamins, but also significantly affect gastrointestinal motor, sensory and secretory functions, intestinal barrier permeability and the regulation of the inflammatory response. The results of recent studies have revealed complex interactions between bile acids and the gut microbiota. In addition, bile acids also play a role of signaling molecules regulating the activity of lipid and glucose metabolic pathways, as well as a role of ligands for transcription factors. Genetic factors associated with the regulation of bile acid synthesis, transport and action may significantly influence gastrointestinal function and predispose to diarrhea resulting from bile acid malabsorption. Methods used in the diagnosis of bile acid malabsorption include 75selenium-homotaurocholic acid test, serum C4 and fibroblast growth factor 19 (FGF19, as well as fecal bile acid levels. The paper presents the latest data on the role of bile acid in the pathogenesis of irritable bowel syndrome, inflammatory bowel diseases and colorectal cancer. Advances in the treatment of disturbances in bile acids absorption and synthesis are also presented. A better understanding of molecular mechanisms regulating bile acid action may have implication for colorectal cancer prevention.

Variations in fluid chemistry and membrane phospholipid fatty acid composition of the bacterial community in a cold storage groundwater system during clogging events

International Nuclear Information System (INIS)

Vetter, Alexandra; Mangelsdorf, Kai; Wolfgramm, Markus; Rauppach, Kerstin; Schettler, Georg; Vieth-Hillebrand, Andrea

2012-01-01

In order to monitor the operating mode of the cold storage of the German Parliament (North German Basin) the fluid chemistry and the phospholipid fatty acid (PLFA) composition of the indigenous microbial community have been monitored from August 2006 to August 2009. During this time two periods of reduced injection (clogging events) characterized by Fe precipitates and microbial biofilms in filters occurred in the injection wells impairing the operating state of the investigated cold storage. The fluid monitoring revealed the presence of sufficient amounts of potential C and energy sources (e.g. DOC and SO 4 2- ) in the process water to sustain microbial life in the cold storage. In times of reduced injection the PLFA inventory of the microbial community differs significantly from times of normal operating phases indicating compositional changes in the indigenous microbial ecosystem. The most affected fatty acids (FAs) are 16:1ω7c (increase), 16:1ω7t (decrease) and 18:1ω7c (increase), interpreted to originate mainly from Fe and S oxidizers, as well as branched FA with 15, 16 and 17 C atoms (decrease) most likely representing sulfate-reducing bacteria (SRB). Based on this variability, PLFA ratios have been created to reflect the increasing dominance of biofilm forming S and Fe oxidizers during the disturbance periods. These ratios are potential diagnostic tools to assess the microbiological contribution to the clogging events and to find appropriate counteractive measures (e.g. mechanical cleaning vs disinfection). The correlation between changes in the PLFA composition and the operational state suggests that microbially mediated processes play a significant role in the observed clogging events in the investigated cold storage.

Niemann-Pick disease, type B with TRAP-positive storage cells and secondary sea blue histiocytosis

Directory of Open Access Journals (Sweden)

R. Saxena

2009-09-01

Full Text Available We present 2 cases of Niemann Pick disease, type B with secondary sea-blue histiocytosis. Strikingly, in both cases the Pick cells were positive for tartrate resistant acid phosphatase, a finding hitherto described only in Gaucher cells. This report highlights the importance of this finding as a potential cytochemical diagnostic pitfall in the diagnosis of Niemann Pick disease.

Diagnosis of Refsum's disease using [1-14C]phytanic acid as substrate

International Nuclear Information System (INIS)

Poulos, A.

1981-01-01

The suitability of using [1- 14 C]phytanic acid as a substrate for the diagnosis of Refsum's disease has been examined. Normal fibroblasts cultured in medium containing low concentrations of foetal calf serum (0.5%) oxidised added [1- 14 C]phytanic acid to 14 CO 2 only slowly up to about 2 days; byond this period a marked stimulation in oxidation was observed. Easily measurable conversion of the radiolabelled acid (from 1.5 to 5.0%) was obtaned by numbers of cells at least one order of magnitude fewer than previously described. Fibroblasts from adult patients with Refsum's disease displayed about 5-10% of the normal mean activity. Small differences in residual activity were observed in the different cell lines. However, no obvious relationship was found between the degree of residual activity, the level of plasma phytanate, and the patient's clinical condition and history. (author)

Recent development and gene therapy for glycogen storage disease type Ia.

Science.gov (United States)

Chou, Janice Y; Kim, Goo-Young; Cho, Jun-Ho

2017-09-01

Glycogen storage disease type Ia (GSD-Ia) is an autosomal recessive metabolic disorder caused by a deficiency in glucose-6-phosphatase-α (G6Pase-α or G6PC) that is expressed primarily in the liver, kidney, and intestine. G6Pase-α catalyzes the hydrolysis of glucose-6-phosphate (G6P) to glucose and phosphate in the terminal step of gluconeogenesis and glycogenolysis, and is a key enzyme for endogenous glucose production. The active site of G6Pase-α is inside the endoplasmic reticulum (ER) lumen. For catalysis, the substrate G6P must be translocated from the cytoplasm into the ER lumen by a G6P transporter (G6PT). The functional coupling of G6Pase-α and G6PT maintains interprandial glucose homeostasis. Dietary therapies for GSD-Ia are available, but cannot prevent the long-term complication of hepatocellular adenoma that may undergo malignant transformation to hepatocellular carcinoma. Animal models of GSD-Ia are now available and are being exploited to both delineate the disease more precisely and develop new treatment approaches, including gene therapy.

Effect of ripeness and postharvest storage on the phenolic profiles of cherries (Prunus avium L.)

DEFF Research Database (Denmark)

Goncalves, B.; Landbo, Anne-Katrine Regel; Knudsen, D.

2004-01-01

temperature) and 1-2 degreesC (cool temperature). Neochlorogenic and p-coumaroylquinic acids were the main hydroxycinnamic acid derivatives, but chlorogenic acid was also identified in all cultivars. The 3-glucoside and 3-rutinoside of cyanidin were the major anthocyanins. Peonidin and pelargonidin 3...... [227 mg/100 g of fresh weight (fw)] and cv. Van the lowest (124 mg/100 g of fw). Phenolic acid contents generally decreased with storage at 1-2 degreesC and increased with storage at 15+/-5 degreesC. Anthocyanin levels increased at both storage temperatures. In cv. Van the anthocyanins increased up...... to 5-fold during storage at 15+/-5 degreesC (from 47 to 230 mg/100 g of fw). Flavonol and flavan-3-ol contents remained quite constant. For all cultivars the levels of phenolic acids were higher in 2001 and the anthocyanin levels were higher in 2002, which suggest a significant influence of climatic...

Bile acid treatment alters hepatic disease and bile acid transport in peroxisome-deficient PEX2 Zellweger mice

NARCIS (Netherlands)

Keane, Megan H.; Overmars, Henk; Wikander, Thomas M.; Ferdinandusse, Sacha; Duran, Marinus; Wanders, Ronald J. A.; Faust, Phyllis L.

2007-01-01

The marked deficiency of peroxisomal organelle assembly in the PEX2(-/-) mouse model for Zellweger syndrome provides a unique opportunity to developmentally and biochemically characterize hepatic disease progression and bile acid products. The postnatal survival of homozygous mutants enabled us to

Neuroimaging of Lipid Storage Disorders

Science.gov (United States)

Rieger, Deborah; Auerbach, Sarah; Robinson, Paul; Gropman, Andrea

2013-01-01

Lipid storage diseases, also known as the lipidoses, are a group of inherited metabolic disorders in which there is lipid accumulation in various cell types, including the central nervous system, because of the deficiency of a variety of enzymes. Over time, excessive storage can cause permanent cellular and tissue damage. The brain is particularly…

Clinical presentation and biochemical findings children with glycogen storage disease type 1A

International Nuclear Information System (INIS)

Saeed, A.; Suleman, H.; Arshad, H.

2015-01-01

To determine the clinical pattern of presentation and biochemical characteristics of glycogen storage disease (GSD) type 1a in children at a tertiary referral centre. Study Design: Descriptive/ cross sectional study. Place and Duration of Study: Department of Pediatric, division of Gastroenterology and Hepatology of the Children's hospital, Lahore over a period of 11 years. Patients and Methods: Confirmed cases of glycogen storage disease (clinical plus biochemical findings consistent with GSD 1a and proven on liver biopsy) were enrolled in this study from neonatal age till 18 years. Data was retrieved from files and electronic record for these cases. Diagnosis was made on the basis of history, clinical findings including hepatomegaly, hypertriglyceridemia, hypercholesterolemia, hypoglycemia and hyperuricemia (if present). Diagnosis was confirmed on liver biopsy. Patients with other storage disorders and benign and malignant tumours were excluded from the study. Results: Total patients included in the study were 360 with male to female ratio of 1.25:1. Median age at the time of diagnosis was 25.6 months (age range from one month to 18 years). Most common presentation was abdominal distension (83%) followed by failure to thrive (69%) and recurrent wheezing and diarrhoea (44%) each. Seizures were present in only 1/3rd of children. Other presentations included vomiting, respiratory distress, altered sensorium, nephrocalcinosis, epistaxis and hypothyroidism. Few patients around 11% presented with acute hepatitis and later were diagnosed as GSD. Significant hepatomegaly was evident in almost all patients but nephromegaly was present in only 5.5% patients. All children had marked hypertriglyceridemia but cholesterol levels were raised in 1/3rd of children. A large majority of children had deranged ALT more than 2 times of normal and around 38% children had marked anemia. Significant hypoglycemia and metabolic acidosis was documented in around 1/3rd of children

Adeno-Associated Virus-Mediated Correction of a Canine Model of Glycogen Storage Disease Type Ia

OpenAIRE

Weinstein, David A.; Correia, Catherine E.; Conlon, Thomas; Specht, Andrew; Verstegen, John; Onclin-Verstegen, Karine; Campbell-Thompson, Martha; Dhaliwal, Gurmeet; Mirian, Layla; Cossette, Holly; Falk, Darin J.; Germain, Sean; Clement, Nathalie; Porvasnik, Stacy; Fiske, Laurie

2010-01-01

This study by the groups of Drs. Barry Byrne and Cathryn Mah at the University of Florida examines the safety and efficacy of AAV-mediated gene delivery in a canine model of glycogen storage disease type Ia (GSDIa). The authors find that intraportal delivery of AAV8 encoding glucose-6-phosphatase-α (G6Pase) followed 20 weeks later by intraportal administration of AAV1 encoding G6Pase led to significant correction of the GSDIa phenotype.

Lipid oxidative changes in traditional dry fermented sausage Petrovská klobása during storage

OpenAIRE

Šojić, Branislav V.; Petrović, Ljiljana S.; Mandić, Anamarija I.; Sedej, Ivana J.; Džinić, Natalija R.; Tomović, Vladimir M.; Jokanović, Marija R.; Tasić, Tatjana A.; Škaljac, Snežana B.; Ikonić, Predrag M.

2014-01-01

The influence of drying and ripeninig conditions (traditional and industrial) in the production of dry fermented sausage Petrovská klobása, on fatty-acid composition and oxidative changes in lipids, during 7 months of storage, was investigated. During the storage period, the sum of unsaturated fatty acids and the content of free fatty acids were significantly higher (p

Storage Time and Urine Biomarker Levels in the ASSESS-AKI Study

Science.gov (United States)

Liu, Kathleen D.; Siew, Edward D.; Reeves, W. Brian; Himmelfarb, Jonathan; Go, Alan S.; Hsu, Chi-yuan; Bennett, Michael R.; Devarajan, Prasad; Ikizler, T. Alp; Kaufman, James S.; Kimmel, Paul L.; Chinchilli, Vernon M.; Parikh, Chirag R.

2016-01-01

Background Although stored urine samples are often used in biomarker studies focused on acute and chronic kidney disease, how storage time impacts biomarker levels is not well understood. Methods 866 subjects enrolled in the NIDDK-sponsored ASsessment, Serial Evaluation, and Subsequent Sequelae in Acute Kidney Injury (ASSESS-AKI) Study were included. Samples were processed under standard conditions and stored at -70°C until analyzed. Kidney injury molecule-1 (KIM-1), neutrophil gelatinase-associated lipocalin (NGAL), interleukin-18 (IL-18), and liver fatty acid binding protein (L-FABP) were measured in urine samples collected during the index hospitalization or an outpatient visit 3 months later. Mixed effects models were used to determine the effect of storage time on biomarker levels and stratified by visit. Results Median storage was 17.8 months (25–75% IQR 10.6–23.7) for samples from the index hospitalization and 14.6 months (IQR 7.3–20.4) for outpatient samples. In the mixed effects models, the only significant association between storage time and biomarker concentration was for KIM-1 in outpatient samples, where each month of storage was associated with a 1.7% decrease (95% CI -3% to -0.3%). There was no relationship between storage time and KIM-1 levels in samples from the index hospitalization. Conclusion There was no significant impact of storage time over a median of 18 months on urine KIM-1, NGAL, IL-18 or L-FABP in hospitalized samples; a statistically significant effect towards a decrease over time was noted for KIM-1 in outpatient samples. Additional studies are needed to determine whether longer periods of storage at -70°C systematically impact levels of these analytes. PMID:27788160

Phase change and heat transfer characteristics of a eutectic mixture of palmitic and stearic acids as PCM in a latent heat storage system

International Nuclear Information System (INIS)

Baran, Guelseren; Sari, Ahmet

2003-01-01

The phase change and heat transfer characteristics of a eutectic mixture of palmitic and stearic acids as phase change material (PCM) during the melting and solidification processes were determined experimentally in a vertical two concentric pipes energy storage system. This study deals with three important subjects. First is determination of the eutectic composition ratio of the palmitic acid (PA) and stearic acid (SA) binary system and measurement of its thermophysical properties by differential scanning calorimetry (DSC). Second is establishment of the phase transition characteristics of the mixture, such as the total melting and solidification temperatures and times, the heat transfer modes in the melted and solidified PCM and the effect of Reynolds and Stefan numbers as initial heat transfer fluid (HTF) conditions on the phase transition behaviors. Third is calculation of the heat transfer coefficients between the outside wall of the HTF pipe and the PCM, the heat recovery rates and heat fractions during the phase change processes of the mixture and also discussion of the effect of the inlet HTF parameters on these characteristics. The DSC results showed that the PA-SA binary system in the mixture ratio of 64.2:35.8 wt% forms a eutectic, which melts at 52.3 deg. C and has a latent heat of 181.7 J g -1 , and thus, these properties make it a suitable PCM for passive solar space heating and domestic water heating applications with respect to climate conditions. The experimental results also indicated that the eutectic mixture of PA-SA encapsulated in the annulus of concentric double pipes has good phase change and heat transfer characteristics during the melting and solidification processes, and it is an attractive candidate as a potential PCM for heat storage in latent heat thermal energy storage systems

The clinicopathology and pathology of selective toxicoses and storage diseases of the nervous system of ruminants in Southern Africa

NARCIS (Netherlands)

Lugt, Jacob Jan van der

2002-01-01

In this study the clinical signs and pathology of five plant poisonings and a mycotoxicosis affecting the nervous system of domestic ruminants in southern Africa are described. For comparative purposes, an inherited storage disease (bèta-mannosidosis) and a drug-induced neurotoxicosis (closantel

[Nonesterified fatty acids and the titrable acidity of breast milk. Consequences for collection conditions in milk bans].

Science.gov (United States)

Luzeau, R; Barrois, V; Odièvre, M

1983-01-01

The study of breast-milk samples, fresh or after storage, shows that the titrable acidity (expressed in degrees Dornic) is directly correlated with their nonesterified fatty acid concentration. Those fresh samples which contain a high activity of lipoprotein lipase can develop in situ lipolysis. The resulting elevated titrable acidity may lead to consider these samples as unsuitable for infant nutrition. These results suggest that collection and storage of breast-milk have to be reassessed in order to avoid in situ lipolysis.

Delivery of acid sphingomyelinase in normal and niemann-pick disease mice using intercellular adhesion molecule-1-targeted polymer nanocarriers.

Science.gov (United States)

Garnacho, Carmen; Dhami, Rajwinder; Simone, Eric; Dziubla, Thomas; Leferovich, John; Schuchman, Edward H; Muzykantov, Vladimir; Muro, Silvia

2008-05-01

Type B Niemann-Pick disease (NPD) is a multiorgan system disorder caused by a genetic deficiency of acid sphingomyelinase (ASM), for which lung is an important and challenging therapeutic target. In this study, we designed and evaluated new delivery vehicles for enzyme replacement therapy of type B NPD, consisting of polystyrene and poly(lactic-coglycolic) acid polymer nanocarriers targeted to intercellular adhesion molecule (ICAM)-1, an endothelial surface protein up-regulated in many pathologies, including type B NPD. Real-time vascular imaging using intravital microscopy and postmortem imaging of mouse organs showed rapid, uniform, and efficient binding of fluorescently labeled ICAM-1-targeted ASM nanocarriers (anti-ICAM/ASM nanocarriers) to endothelium after i.v. injection in mice. Fluorescence microscopy of lung alveoli actin, tissue histology, and 125I-albumin blood-to-lung transport showed that anti-ICAM nanocarriers cause neither detectable lung injury, nor abnormal vascular permeability in animals. Radioisotope tracing showed rapid disappearance from the circulation and enhanced accumulation of anti-ICAM/125I-ASM nanocarriers over the nontargeted naked enzyme in kidney, heart, liver, spleen, and primarily lung, both in wild-type and ASM knockout mice. These data demonstrate that ICAM-1-targeted nanocarriers may enhance enzyme replacement therapy for type B NPD and perhaps other lysosomal storage disorders.

Effect of the rooster semen enrichment with oleic acid on the quality of semen during chilled storage.

Science.gov (United States)

Eslami, M; Ghaniei, A; Mirzaei Rad, H

2016-06-01

Liquid storage of avian spermatozoa is currently being employed in programs utilizing the artificial insemination to optimize the management of genetically superior males. It is mandatory to use efficient semen storage techniques in order to prevent the reduction of the fertilizing ability of stored semen. The present study was designated to evaluate the effect of oleic acid on rooster semen quality stored at 4°C for 48 h. Semen was collected from 10 roosters twice a week. Good quality ejaculates were pooled and after dilution, the semen was enriched with 0 (control), 0.125 (O 0.125), 0.25 (O 0.25), 0.5 (O 0.5), and 1 (O1) millimolar oleate. Forward progressive motility and viability of spermatozoa were evaluated at 0, 24, and 48 h. Moreover, malondialdehyde (MDA) and total antioxidant activity (AOA) levels were measured in seminal plasma and spermatozoa at the mentioned time points. Motility was 80.33 ± 1.45, 80.00 ± 2.08, and 66.00 ± 2.30% at 24 h and 56.33 ± 1.45, 57.33 ± 2.18, and 41.33 ± 2.02% at 48 h in O 0.125, O 0.25, and control, respectively (P semen enrichment with low doses of oleate would exert beneficial effects on the quality of semen during cooled storage. © 2016 Poultry Science Association Inc.

Antifibrinolytic amino acids for upper gastrointestinal bleeding in people with acute or chronic liver disease.

Science.gov (United States)

Martí-Carvajal, Arturo J; Solà, Ivan

2015-06-09

Upper gastrointestinal bleeding is one of the most frequent causes of morbidity and mortality in the course of liver cirrhosis. People with liver disease frequently have haemostatic abnormalities such as hyperfibrinolysis. Therefore, antifibrinolytic amino acids have been proposed to be used as supplementary interventions alongside any of the primary treatments for upper gastrointestinal bleeding in people with liver diseases. This is an update of this Cochrane review. To assess the beneficial and harmful effects of antifibrinolytic amino acids for upper gastrointestinal bleeding in people with acute or chronic liver disease. We searched The Cochrane Hepato-Biliary Controlled Trials Register (February 2015), Cochrane Central Register of Controlled Trials (CENTRAL) (Issue 2 of 12, 2015), MEDLINE (Ovid SP) (1946 to February 2015), EMBASE (Ovid SP) (1974 to February 2015), Science Citation Index EXPANDED (1900 to February 2015), LILACS (1982 to February 2015), World Health Organization Clinical Trials Search Portal (accessed 26 February 2015), and the metaRegister of Controlled Trials (accessed 26 February 2015). We scrutinised the reference lists of the retrieved publications. Randomised clinical trials irrespective of blinding, language, or publication status for assessment of benefits and harms. Observational studies for assessment of harms. We planned to summarise data from randomised clinical trials using standard Cochrane methodologies and assessed according to the GRADE approach. We found no randomised clinical trials assessing antifibrinolytic amino acids for treating upper gastrointestinal bleeding in people with acute or chronic liver disease. We did not identify quasi-randomised, historically controlled, or observational studies in which we could assess harms. This updated Cochrane review identified no randomised clinical trials assessing the benefits and harms of antifibrinolytic amino acids for upper gastrointestinal bleeding in people with acute or

Bile acid changes after high-dose ursodeoxycholic acid treatment in primary sclerosing cholangitis: relation to disease progression

Science.gov (United States)

Sinakos, Emmanouil; Marschall, Hanns-Ulrich; Kowdley, Kris V.; Befeler, Alex; Keach, Jill; Lindor, Keith

2010-01-01

High-dose (28-30mg/kg/day) ursodeoxycholic acid (UDCA) treatment improves serum liver tests in patients with primary sclerosing cholangitis (PSC) but does not improve survival and is associated with increased rates of serious adverse events. The mechanism for the latter undesired effect remains unclear. High-dose UDCA could result in the production of hepatotoxic bile acids, such as lithocholic acid (LCA), due to limited small bowel absorption of UDCA and conversion of UDCA by bacteria in the colon. We determined the serum bile acid composition in 56 patients with PSC previously enrolled in a randomized, double-blind controlled trial of high dose UDCA versus placebo. Samples for analysis were obtained at baseline and at the end of treatment. The mean changes in UDCA (16.86 vs 0.05 μmol/L) and total bile acid (17.21 vs −0.55 μmol/L) levels were significantly higher in the UDCA group (n=29) compared to placebo (n=27) when pretreatment levels were compared (pacid (CA), deoxycholic acid (DCA) and chenodeoxycholic acid (CDCA). Patients (n=9) in the UDCA group who reached clinical endpoints of disease progression (development of cirrhosis, varices, liver transplantation or death) tend to have greater increase in their post-treatment total bile acid levels (34.99 vs 9.21 μmol/L) (pacid pool including lithocholic acid. PMID:20564380

Bile Acids in the Treatment of Cardiometabolic Diseases.